Project: Ghana Emergency Medicine Collaborative, 2013 Document Title: Hematologic and Oncologic...

Project: Ghana Emergency Medicine Collaborative, 2013

Document Title: Hematologic and Oncologic Emergencies

Author(s): Joe Lex, MD, FACEP, FAAEM, MAAEM (Temple University)

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Hematologic and Oncologic Hematologic and Oncologic EmergenciesEmergencies

Joe Lex, MD, FACEP, FAAEM, MAAEMJoe Lex, MD, FACEP, FAAEM, MAAEMAssociate Professor of Emergency Medicine, Associate Professor of Emergency Medicine,

Department of Emergency MedicineDepartment of Emergency Medicine

Temple University School of MedicineTemple University School of Medicine

Philadelphia, PA USAPhiladelphia, PA USA

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MenuMenu

• TransfusionsTransfusions

• Bleeding disordersBleeding disorders

• Platelet disordersPlatelet disorders

• Red cell disordersRed cell disorders

• White cell disordersWhite cell disorders

• Oncologic emergenciesOncologic emergencies

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QuestionsQuestions

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Who Needs Blood?Who Needs Blood?

• Most patients uncomfortable if Most patients uncomfortable if hemoglobin concentration <7 g/dLhemoglobin concentration <7 g/dL

• Patients with heart or pulmonary Patients with heart or pulmonary disease may need hemoglobin disease may need hemoglobin concentration of concentration of 10 g/dL to 10 g/dL to safeguard adequate oxygen safeguard adequate oxygen transporttransport

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WhatWhat’’s Available? s Available?

• Packed Red Blood Cells: Packed Red Blood Cells: centrifuged whole blood with 80% centrifuged whole blood with 80% of plasma removedof plasma removed

• Adult: 1 unit PRBC raises Adult: 1 unit PRBC raises hemoglobin by 1 gm/dLhemoglobin by 1 gm/dL

• Child: Each mL/kg raises Child: Each mL/kg raises hemoglobin by 1 gm/dLhemoglobin by 1 gm/dL

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WhatWhat’’s Available? s Available?

• Platelets: shelf life ~5 daysPlatelets: shelf life ~5 days– ABO / Rh checks are neededABO / Rh checks are needed

– ““Six packSix pack”” raises count by ~30,000 raises count by ~30,000

• Fresh Frozen Plasma: ~1 yearFresh Frozen Plasma: ~1 year– 1 unit / mL of each coagulation factor1 unit / mL of each coagulation factor

– No cellular componentsNo cellular components

– 10–15 mL / kg (3–4 250 mL bags)10–15 mL / kg (3–4 250 mL bags)

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Special NeedsSpecial Needs

• All blood products now leukocyte-All blood products now leukocyte-depleteddepleted

• Washed cell products in patients Washed cell products in patients with confirmed deficiency of IgAwith confirmed deficiency of IgA

• Irradiated cell products used to Irradiated cell products used to prevent graft-versus-host reactions prevent graft-versus-host reactions in immunosuppressed patientsin immunosuppressed patients

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Acute Intravascular Hemolytic Acute Intravascular Hemolytic Transfusion ReactionTransfusion Reaction

• Typically due to ABO incompatibility Typically due to ABO incompatibility (clerical error)(clerical error)

• Intravascular lysis of donor RBCsIntravascular lysis of donor RBCs

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• Sudden fever, chills, headache, Sudden fever, chills, headache, flushing, low back pain, nausea, flushing, low back pain, nausea, tachycardia, hypotensiontachycardia, hypotension

• Shortly after transfusion startsShortly after transfusion starts

• Hemoglobinuria, hemoglobinemiaHemoglobinuria, hemoglobinemia

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• Stop infusionStop infusion

• Direct Coombs on recipientDirect Coombs on recipient’’s blood s blood detects host antibodiesdetects host antibodies

• Look at plasma: if red…Look at plasma: if red…

• Look at urine: if red…Look at urine: if red…

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• CBC, chemistries, liver panel, CBC, chemistries, liver panel, coagulation profile, haptoglobin, coagulation profile, haptoglobin, lactate dehydrogenase (LDH)lactate dehydrogenase (LDH)

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• Send bag to blood bankSend bag to blood bank

• IV fluids (new tubing)IV fluids (new tubing)

• Consider furosemide, mannitolConsider furosemide, mannitol

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Febrile NonhemolyticFebrile Nonhemolytic

• Multiple prior transfusions or Multiple prior transfusions or multiparous womenmultiparous women

• Fever, chills shortly after startFever, chills shortly after start

• Same workup as acute Same workup as acute intravascular hemolytic reactionintravascular hemolytic reaction

• Diagnosis of exclusionDiagnosis of exclusion

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• Stop transfusionStop transfusion

• IV fluid through new tubingIV fluid through new tubing

• AntipyreticAntipyretic

• Broad-spectrum antibiotic(s)Broad-spectrum antibiotic(s)

• Future transfusions: probably Future transfusions: probably leukoreducedleukoreduced

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• MILD: if patient responds to MILD: if patient responds to antihistamine, may resume antihistamine, may resume transfusiontransfusion

• SEVERE: antihistamine, steroids, SEVERE: antihistamine, steroids, epinephrine, IVFepinephrine, IVF

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Massive TransfusionMassive Transfusion

• Hyperkalemia or hypokalemiaHyperkalemia or hypokalemia

• Citrate breakdown into bicarbonate Citrate breakdown into bicarbonate metabolic alkalosis metabolic alkalosis

• Citrate / serum calcium complex Citrate / serum calcium complex hypocalcemiahypocalcemia

• CoagulopathyCoagulopathy

• Hypothermia from cold bloodHypothermia from cold blood

• Volume overloadVolume overload 20

Delayed HemolyticDelayed Hemolytic

• Extravascular: antibodies react to Extravascular: antibodies react to non-ABO antigensnon-ABO antigens

• Antibody-coated RBCs removed Antibody-coated RBCs removed from circulation by liver and spleen from circulation by liver and spleen before they are lysed before they are lysed extravascular extravascular no no hemoglobinemia, hemoglobinuria hemoglobinemia, hemoglobinuria

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Delayed HemolyticDelayed Hemolytic

• Typically asymptomatic, may have Typically asymptomatic, may have feverfever

• Same workup as acute Same workup as acute intravascular hemolytic reaction intravascular hemolytic reaction

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TRALITRALI

• Transfusion-associated acute lung Transfusion-associated acute lung injury (TRALI)injury (TRALI)

• Increasingly recognized event Increasingly recognized event

• Acute respiratory distress, often Acute respiratory distress, often associated with fever, non-associated with fever, non-cardiogenic pulmonary edema, and cardiogenic pulmonary edema, and hypotensionhypotension

• ~1 in 2000 transfusions~1 in 2000 transfusions23

Transfusion-Associated Graft-Transfusion-Associated Graft-versus-Host Diseaseversus-Host Disease

• Donor lymphocytes recognize Donor lymphocytes recognize immunocompromised recipient as immunocompromised recipient as foreign, attack host tissuesforeign, attack host tissues

• Usually 4–10d post transfusionUsually 4–10d post transfusion

• Fever, N-V, rash, diarrheaFever, N-V, rash, diarrhea

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Transfusion-Associated Graft-Transfusion-Associated Graft-versus-Host Diseaseversus-Host Disease

• Same workup as acute Same workup as acute intravascular hemolytic reactionintravascular hemolytic reaction

• No effective treatment: possibly No effective treatment: possibly stem cell transplantstem cell transplant

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Disease TransmissionDisease Transmission

• HIV: 1 in 2,000,000HIV: 1 in 2,000,000

• Hepatitis B: 1 in 137,000Hepatitis B: 1 in 137,000

• Hepatitis C: 1 in 1,000,000Hepatitis C: 1 in 1,000,000

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Other ComplicationsOther Complications

• Hypothermia from cold bloodHypothermia from cold blood

• Volume overloadVolume overload

• Breakdown of citrate into Breakdown of citrate into bicarbonate bicarbonate metabolic alkalosis metabolic alkalosis

• Citrate / serum calcium complex Citrate / serum calcium complex hypocalcemiahypocalcemia

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8.8.Your patient is profoundly anemic and Your patient is profoundly anemic and you believe she would benefit from a you believe she would benefit from a blood transfusion. In weighing benefits blood transfusion. In weighing benefits against risks, you tell her that the most against risks, you tell her that the most common adverse effect is: common adverse effect is:

a.a. hepatitis C transmission.hepatitis C transmission.b.b. hepatitis B transmission.hepatitis B transmission.c.c. human immunodeficiency virus (HIV) human immunodeficiency virus (HIV)

transmission.transmission.d.d. febrile nonhemolytic reaction. febrile nonhemolytic reaction. e.e. graft versus host reaction.graft versus host reaction.

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8.8.Your patient is profoundly anemic and Your patient is profoundly anemic and you believe she would benefit from a you believe she would benefit from a blood transfusion. In weighing benefits blood transfusion. In weighing benefits against risks, you tell her that the most against risks, you tell her that the most common adverse effect is: common adverse effect is:

a.a. hepatitis C transmission.hepatitis C transmission.b.b. hepatitis B transmission.hepatitis B transmission.c.c. human immunodeficiency virus (HIV) human immunodeficiency virus (HIV)

transmission.transmission.d.d. febrile nonhemolytic reaction. febrile nonhemolytic reaction. e.e. graft versus host reaction.graft versus host reaction.

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8.8. Most common transfusion adverseMost common transfusion adverse

Febrile non-hemolytic reaction is Febrile non-hemolytic reaction is estimated to occur once for every 200 estimated to occur once for every 200 units transfused. During the units transfused. During the transfusion or within a few hours after transfusion or within a few hours after its completion, the patient has a its completion, the patient has a temperature elevation of at least 1°C temperature elevation of at least 1°C and usually has chills. The usual and usually has chills. The usual cause of this febrile reaction is an cause of this febrile reaction is an antigen-antibody reaction involving the antigen-antibody reaction involving the plasma, platelets, or white blood cells plasma, platelets, or white blood cells that are passively transfused to the that are passively transfused to the recipient along with the RBCs.recipient along with the RBCs. 30

8.8. Most common transfusion adverseMost common transfusion adverse

Once a febrile transfusion reaction is Once a febrile transfusion reaction is recognized, the current transfusion recognized, the current transfusion should be terminated because there is should be terminated because there is not sufficient clinical evidence to not sufficient clinical evidence to differentiate the simple febrile differentiate the simple febrile nonhemolytic reaction from the more nonhemolytic reaction from the more serious immediate hemolytic reaction.serious immediate hemolytic reaction.

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Thrombus FormationThrombus Formation

• Damaged epithelium Damaged epithelium collagen collagen and tissue factor (TF) exposed to and tissue factor (TF) exposed to flowing blood flowing blood adherence / adherence / activation of platelets (relies on von activation of platelets (relies on von Willebrand Factor [vWF])Willebrand Factor [vWF])

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• Tissue factor activates coagulation Tissue factor activates coagulation cascade cascade generates thrombin generates thrombin converts fibrinogen to fibrin converts fibrinogen to fibrin forms long, cross-linking strands forms long, cross-linking strands that trap platelets and RBCs to that trap platelets and RBCs to form insoluble clotform insoluble clot

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• Thrombin also triggers tissue Thrombin also triggers tissue plasminogen activator plasminogen activator activates activates plasminplasmin

• Plasmin, anti-thrombin III, protein C Plasmin, anti-thrombin III, protein C & protein S cause fibrinolysis& protein S cause fibrinolysis

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WarfarinWarfarin

• Inhibits Vitamin K-dependent Inhibits Vitamin K-dependent coagulation factors II, VII, IX, Xcoagulation factors II, VII, IX, X– Half-lives 7h (FVII) to 50h (FII)Half-lives 7h (FVII) to 50h (FII)

• Inhibits Vitamin K-dependent Inhibits Vitamin K-dependent anticoagulants Protein C & Santicoagulants Protein C & S– Half-lives up to 40hHalf-lives up to 40h

• 11stst few days: may be procoaguable few days: may be procoaguable

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Warfarin Overdose: No BleedWarfarin Overdose: No Bleed

• INR <5: omit next warfarin or INR <5: omit next warfarin or dosedose

• INR 5 – 9: omit next 1-2 doses, INR 5 – 9: omit next 1-2 doses, give oral vitamin K (1 – 2.5 mg)give oral vitamin K (1 – 2.5 mg)

• INR >9: omit warfarin, give oral INR >9: omit warfarin, give oral vitamin K (2.5 – 5 mg)vitamin K (2.5 – 5 mg)

• Avoid subcutaneous vitamin KAvoid subcutaneous vitamin K

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Warfarin Overdose: BleedWarfarin Overdose: Bleed

• Vitamin K 10 mg slow IV infusionVitamin K 10 mg slow IV infusion

• Fresh frozen plasma (FFP): start Fresh frozen plasma (FFP): start with 4 – 6 unitswith 4 – 6 units– Each ml of FFP contains 1unit of Each ml of FFP contains 1unit of

each coagulation factoreach coagulation factor

– INR will not correct beyond ~1.5INR will not correct beyond ~1.5

• Prothrombin complex concentrateProthrombin complex concentrate– Rapid, expensive, procoaguabilityRapid, expensive, procoaguability

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Heparin OverdoseHeparin Overdose

• Heparin activates antithrombin IIIHeparin activates antithrombin III

• Short half-life: stopping drip will Short half-life: stopping drip will normalize in few hoursnormalize in few hours

• Can reverse with protamine sulfate: Can reverse with protamine sulfate: 1mg IV for every 100u heparin 1mg IV for every 100u heparin unfractionated given in prior 2 hrsunfractionated given in prior 2 hrs– Works a little for LMWHWorks a little for LMWH

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Liver DiseaseLiver Disease

• Liver synthesizes nearly all clotting Liver synthesizes nearly all clotting factorsfactors

PT/INR do not correlate well PT/INR do not correlate well with risk of bleedingwith risk of bleeding

• Can reverse with FFP if active Can reverse with FFP if active bleeding, require invasive bleeding, require invasive procedureprocedure

• ± vitamin K± vitamin K40

HemophiliasHemophilias

• Inherited: sex-linked recessiveInherited: sex-linked recessive

• Factor VIII: hemophilia A (more Factor VIII: hemophilia A (more common)common)

• Factor IX: hemophilia BFactor IX: hemophilia B

• Clinically indistinguishableClinically indistinguishable

• PTT elevated in bothPTT elevated in both

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Clinical SeverityClinical Severity

• Related to activity level of factorRelated to activity level of factor

<1% - severe <1% - severe frequent frequent spontaneous bleedsspontaneous bleeds

1 – 5% - moderate 1 – 5% - moderate occasional occasional spontaneous, lots in traumaspontaneous, lots in trauma

>5% - mild >5% - mild no spontaneous, no spontaneous, excessive in trauma, surgeryexcessive in trauma, surgery

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• Vast majority in ED have known Vast majority in ED have known disease, present with complicationsdisease, present with complications

• Most common: intramuscular, intra-Most common: intramuscular, intra-articular bleedsarticular bleeds

PresentationPresentation

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Treatment: Based on ComplaintTreatment: Based on Complaint

• Minor bleeding: replace 25%Minor bleeding: replace 25%

• Severe bleeding: replace 50%Severe bleeding: replace 50%

• Life-threatening: replace 100%Life-threatening: replace 100%

• FVIII: 1U/kg FVIII: 1U/kg plasma level ~2% plasma level ~2%

• FIX: 1U/kg FIX: 1U/kg plasma level ~1% plasma level ~1%

• In ED, assume baseline plasma In ED, assume baseline plasma level to be zerolevel to be zero

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Replacement FactorsReplacement Factors

• Can be recombinant or derived Can be recombinant or derived from human plasmafrom human plasma

• Hemophilia A: mild to moderate Hemophilia A: mild to moderate start with DDAVPstart with DDAVP– Causes release of vWF and FVIII Causes release of vWF and FVIII

from endothelial cellsfrom endothelial cells

• Arthrocentesis rarely indicatedArthrocentesis rarely indicated

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5.5.The initial dose of factor VIII required The initial dose of factor VIII required for a 60-kg male with severe for a 60-kg male with severe hemophilia A in whom you suspect a hemophilia A in whom you suspect a ruptured spleen is:ruptured spleen is:

a.a. 1,500 units1,500 unitsb.b. 2,850 units2,850 unitsc.c. 3,000 units3,000 unitsd.d. 6,000 units6,000 unitse.e. 5,700 units 5,700 units

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5.5.The initial dose of factor VIII required The initial dose of factor VIII required for a 60-kg male with severe for a 60-kg male with severe hemophilia A in whom you suspect a hemophilia A in whom you suspect a ruptured spleen is:ruptured spleen is:

a.a. 1,500 units1,500 unitsb.b. 2,850 units2,850 unitsc.c. 3,000 units3,000 unitsd.d. 6,000 units6,000 unitse.e. 5,700 units 5,700 units

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5.5. … …initial dose of factor VIII … severe initial dose of factor VIII … severe hemophilia A … ruptured spleen … 3,000 hemophilia A … ruptured spleen … 3,000 units.units.

A 60-kg patient with a life threatening A 60-kg patient with a life threatening hemorrhage who requires 100% hemorrhage who requires 100% correction will need 50 mL/kg = 3000 correction will need 50 mL/kg = 3000 units of factor VIII units of factor VIII

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von Willebrand Diseasevon Willebrand Disease

• Mucocutaneous bleeding more Mucocutaneous bleeding more likelylikely

• Hemarthrosis less likelyHemarthrosis less likely

• Humate F®: FVIII-vWF concentrateHumate F®: FVIII-vWF concentrate

• DDAVP only effective in Type 1DDAVP only effective in Type 1

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15.15.A 38-year-old woman has von A 38-year-old woman has von WillebrandWillebrand’’s disease, Type I. She s disease, Type I. She complains of blood-tinged emesis and complains of blood-tinged emesis and epigastric pain. Her stool tests weakly epigastric pain. Her stool tests weakly positive for blood. Appropriate initial positive for blood. Appropriate initial therapy includes:therapy includes:

a.a. vitamin K.vitamin K.b.b. 6 units platelet concentrate.6 units platelet concentrate.c.c. factor IX concentrate.factor IX concentrate.d.d. desmopressin (DDAVP).desmopressin (DDAVP).e.e. plasmapheresis.plasmapheresis.

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15.15.A 38-year-old woman has von A 38-year-old woman has von WillebrandWillebrand’’s disease, Type I. She s disease, Type I. She complains of blood-tinged emesis and complains of blood-tinged emesis and epigastric pain. Her stool tests weakly epigastric pain. Her stool tests weakly positive for blood. Appropriate initial positive for blood. Appropriate initial therapy includes:therapy includes:

a.a. vitamin K.vitamin K.b.b. 6 units platelet concentrate.6 units platelet concentrate.c.c. factor IX concentrate.factor IX concentrate.d.d. desmopressin (DDAVP).desmopressin (DDAVP).e.e. plasmapheresis.plasmapheresis.

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15.15.von Willebrandvon Willebrand’’s disease, Type I ... s disease, Type I ... blood-tinged emesis … initial therapy…blood-tinged emesis … initial therapy…

Treatment of von WillebrandTreatment of von Willebrand’’s disease s disease depends on the type of disease that is depends on the type of disease that is present and the severity of bleeding. present and the severity of bleeding. Desmopressin (DDAVP) treatment has Desmopressin (DDAVP) treatment has benefit in patients with mild to benefit in patients with mild to moderately severe von Willebrandmoderately severe von Willebrand’’s s disease, but should be given in disease, but should be given in consultation with a hematologist. consultation with a hematologist. Factor VIII (cryoprecipitate) or fresh Factor VIII (cryoprecipitate) or fresh frozen plasma may be used in patients frozen plasma may be used in patients with severe bleeding. with severe bleeding.

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Disseminated Intravascular Disseminated Intravascular Coagulation (DIC)Coagulation (DIC)

• Many potential causes: infection, Many potential causes: infection, malignancy, trauma, OBmalignancy, trauma, OB

• Loss of hemostasis regulatory Loss of hemostasis regulatory mechanismsmechanisms

• Thrombin overproduced Thrombin overproduced promotes clottingpromotes clotting

• Fibrin obstructs small vesselsFibrin obstructs small vessels

• Consumed platelets and factorsConsumed platelets and factors54


• May be lab abnormalities only: no May be lab abnormalities only: no overt clinical signsovert clinical signs

• Bleeding, organ dysfunction Bleeding, organ dysfunction possiblepossible

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• CBC: thrombocytopenia, possible CBC: thrombocytopenia, possible anemiaanemia

• Blood smear: schistocytesBlood smear: schistocytes

• PT/INR, PTT: prolongedPT/INR, PTT: prolonged

• D-dimer/FSP: elevatedD-dimer/FSP: elevated

• Fibrinogen: diminishedFibrinogen: diminished

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TreatmentTreatment

• Treat underlying causeTreat underlying cause

• Bleeding-predominant: FFP, Bleeding-predominant: FFP, platelets, cryoprecipitateplatelets, cryoprecipitate

• Clotting-predominant: heparinClotting-predominant: heparin– More common in chronic DICMore common in chronic DIC

– Not appropriate for acute DICNot appropriate for acute DIC

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3.3.The most helpful lab study in diagnosing The most helpful lab study in diagnosing disseminated intravascular coagulation is disseminated intravascular coagulation is the:the:

a.a. D-dimer, which is elevated.D-dimer, which is elevated.

b.b. partial thromboplastin time (PTT), which partial thromboplastin time (PTT), which is decreased.is decreased.

c.c. fibrinogen level, which is elevated.fibrinogen level, which is elevated.

d.d. prothrombin time, which is prolonged.prothrombin time, which is prolonged.

e.e. fibrin degradation products (FDP), which fibrin degradation products (FDP), which are diminished.are diminished.

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3.3.The most helpful lab study in diagnosing The most helpful lab study in diagnosing disseminated intravascular coagulation is disseminated intravascular coagulation is the:the:

a.a. D-dimer, which is elevated.D-dimer, which is elevated.

b.b. partial thromboplastin time (PTT), which partial thromboplastin time (PTT), which is decreased.is decreased.

c.c. fibrinogen level, which is elevated.fibrinogen level, which is elevated.

d.d. prothrombin time, which is prothrombin time, which is prolonged.prolonged.

e.e. fibrin degradation products (FDP), which fibrin degradation products (FDP), which are diminished.are diminished. 59

3.3.Useful labs in DICUseful labs in DIC

MOST USEFULMOST USEFUL::• PT / INR – PT / INR – • Platelet count – usually Platelet count – usually • Fibrinogen level – Fibrinogen level – HELPFULHELPFUL::• aPTT – usually aPTT – usually • Thrombin clot time – Thrombin clot time – • Fragmented RBCs – presentFragmented RBCs – present• FDP and D-dimers – FDP and D-dimers –

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10.10.The cornerstone of Emergency The cornerstone of Emergency Department management of DIC is:Department management of DIC is:

a.a. hemodynamic stabilization and hemodynamic stabilization and treatment of the underlying disorder.treatment of the underlying disorder.

b.b. rapid correction of thrombocytopenia.rapid correction of thrombocytopenia.c.c. aggressive resuscitation with colloid.aggressive resuscitation with colloid.d.d. pan-culture and broad-spectrum pan-culture and broad-spectrum

antibiotic coverage.antibiotic coverage.e.e. rapid intubation and hyperventilation.rapid intubation and hyperventilation.

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10.10.The cornerstone of Emergency The cornerstone of Emergency Department management of DIC is:Department management of DIC is:

a.a. hemodynamic stabilization and hemodynamic stabilization and treatment of the underlying treatment of the underlying disorder.disorder.

b.b. rapid correction of thrombocytopenia.rapid correction of thrombocytopenia.c.c. aggressive resuscitation with colloid.aggressive resuscitation with colloid.d.d. pan-culture and broad-spectrum pan-culture and broad-spectrum

antibiotic coverage.antibiotic coverage.e.e. rapid intubation and hyperventilation.rapid intubation and hyperventilation.

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10.10.Cornerstone of managing DICCornerstone of managing DIC

The primary cause of the DIC needs to be The primary cause of the DIC needs to be determined and treated. The high determined and treated. The high mortality rate in severe DIC is primarily mortality rate in severe DIC is primarily due to the underlying disorder. Many due to the underlying disorder. Many patients with DIC require no specific patients with DIC require no specific therapy if there is no evidence of therapy if there is no evidence of bleeding, or if thrombosis and laboratory bleeding, or if thrombosis and laboratory studies are not deteriorating. The first studies are not deteriorating. The first principle of management is to stabilize principle of management is to stabilize the patient hemodynamically, providing the patient hemodynamically, providing oxygen, fluids, and life support as oxygen, fluids, and life support as needed.needed.

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Platelet DisordersPlatelet Disorders

platelets = thrombocytopeniaplatelets = thrombocytopenia1111 productionproduction

2.2. Splenic sequestrationSplenic sequestration

1111 destruction: drugs, infection, destruction: drugs, infection, DIC, ITP, TTPDIC, ITP, TTP

4.4. Dilution: massive blood Dilution: massive blood transfusiontransfusion

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PlateletsPlatelets

• Platelet life-span: 10 daysPlatelet life-span: 10 days

• Each day marrow produces / Each day marrow produces / releases ~10% of plateletsreleases ~10% of platelets

• Platelet count <20,000: Platelet count <20,000: spontaneous bleedspontaneous bleed

• Platelet count >50,000: minimal Platelet count >50,000: minimal needed to avoid bleeding in trauma needed to avoid bleeding in trauma / surgery/ surgery

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PlateletsPlatelets

• Aspirin blocks COX receptors for Aspirin blocks COX receptors for life of plateletlife of platelet

• Withhold aspirin for 1 day Withhold aspirin for 1 day ~25,000 active platelets~25,000 active platelets

• Withhold aspirin for 2 days Withhold aspirin for 2 days ~50,000 active platelets~50,000 active platelets

• NSAIDs block COX receptors for NSAIDs block COX receptors for life of NSAIDlife of NSAID (~4 – 8 hours) (~4 – 8 hours)

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Thrombotic Thrombocytopenic Thrombotic Thrombocytopenic Purpura (TTP)Purpura (TTP)

Classic Pentad in 40%Classic Pentad in 40%1.1. Microangiopathic hemolysisMicroangiopathic hemolysis

2.2. ThrombocytopeniaThrombocytopenia

3.3. Neurologic symptomsNeurologic symptoms

4.4. FeverFever

5.5. Renal dysfunction (usually mild)Renal dysfunction (usually mild)

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Thrombotic Thrombocytopenic Thrombotic Thrombocytopenic Purpura (TTP)Purpura (TTP)

More Common Triad in 75%More Common Triad in 75%1.1. Microangiopathic hemolysisMicroangiopathic hemolysis

2.2. Thrombocytopenic purpuraThrombocytopenic purpura

3.3. Neurologic symptomsNeurologic symptoms

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• More common in women, adults in More common in women, adults in 44thth decade of life decade of life

• 40% have URI / flu-like symptoms 40% have URI / flu-like symptoms prior to presentationprior to presentation

• Fatigue, malaiseFatigue, malaise

• FeverFever

• Neurologic symptoms: headache, Neurologic symptoms: headache, confusion, seizure, stroke, comaconfusion, seizure, stroke, coma

70

DiagnosisDiagnosis

• Peripheral blood: schistocytesPeripheral blood: schistocytes

• CBC: anemia, thrombocytopeniaCBC: anemia, thrombocytopenia

• LDH: markedly LDH: markedly (ischemia, (ischemia, hemolysis)hemolysis)

• Creatinine: Creatinine: • Urine: blood, protein, castsUrine: blood, protein, casts

• PT/PTT typically normalPT/PTT typically normal

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TreatmentTreatment

• Plasma exchange: very effectivePlasma exchange: very effective

• Platelet transfusion Platelet transfusion CONTRA-CONTRA-INDICATEDINDICATED ( ( thrombus thrombus formation)formation)

• Last resort: steroids, splenectomyLast resort: steroids, splenectomy

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6.6.A 44-year-old woman with a history of TTP, A 44-year-old woman with a history of TTP, in remission for 30 days, presents to the ED in remission for 30 days, presents to the ED complaining of lethargy. Laboratory results complaining of lethargy. Laboratory results would likely show:would likely show:

1a1a LDH, LDH, reticulocyte count, reticulocyte count, red red blood cell count.blood cell count.1b1b LDH, LDH, reticulocyte count, reticulocyte count, creatinine.creatinine.1c1c platelet count, platelet count, red blood cell red blood cell count, count, reticulocyte count. reticulocyte count.1d1d platelet count, platelet count, reticulocyte reticulocyte count, count, LDH. LDH.

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6.6.A 44-year-old woman with a history of TTP, A 44-year-old woman with a history of TTP, in remission for 30 days, presents to the ED in remission for 30 days, presents to the ED complaining of lethargy. Laboratory results complaining of lethargy. Laboratory results would likely show:would likely show:

1a1a LDH, LDH, reticulocyte count, reticulocyte count, red red blood cell count.blood cell count.1b1b LDH, LDH, reticulocyte count, reticulocyte count, creatinine.creatinine.1c1c platelet count, platelet count, red blood cell red blood cell count, count, reticulocyte count. reticulocyte count.1d1d platelet count, platelet count, reticulocyte reticulocyte count, count, LDH. LDH.

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6.6.TTP – Laboratory resultsTTP – Laboratory results

In TTP, laboratory studies will show an In TTP, laboratory studies will show an anemia of variable degree, anemia of variable degree, LDH, LDH, reticulocytosis, reticulocytosis, indirect bilirubin, indirect bilirubin, negative Coombsnegative Coombs’’ test, and test, and schistocytes on peripheral smear. schistocytes on peripheral smear. Thrombocytopenia is often severe with Thrombocytopenia is often severe with the count <20,000/mL in 50% of the count <20,000/mL in 50% of patients. BUN and creatinine are patients. BUN and creatinine are typically typically . .

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Hemolytic Uremic Syndrome (HUS)Hemolytic Uremic Syndrome (HUS)

• Similar to TTP, but worse renal Similar to TTP, but worse renal dysfunction, less severe neurologic dysfunction, less severe neurologic symptomssymptoms– ~60% require hemodialysis~60% require hemodialysis

• ““TypicalTypical”” or or ““childhoodchildhood””: follows : follows acute bloody diarrheaacute bloody diarrhea– Most common: Most common: E coli O157:H7E coli O157:H7

– Usually 5 years and youngerUsually 5 years and younger76

TreatmentTreatment

• Plasma exchange not as effective Plasma exchange not as effective as with TTPas with TTP

• Avoid platelet transfusions as with Avoid platelet transfusions as with TTPTTP

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1.1. A patient with anemia, thrombocytopenia, A patient with anemia, thrombocytopenia, renal failure, normal coagulation tests and a renal failure, normal coagulation tests and a clear sensorium probably has:clear sensorium probably has:

a.a. idiopathic thrombocytopenic anemia.idiopathic thrombocytopenic anemia.

b.b. thrombotic thrombocytopenic purpura.thrombotic thrombocytopenic purpura.

c.c. hemolytic-uremic syndrome.hemolytic-uremic syndrome.

d.d. disseminated intravascular coagulation. disseminated intravascular coagulation.

e.e. autoimmune hemolytic anemia.autoimmune hemolytic anemia.

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1.1.A patient with anemia, thrombocytopenia, A patient with anemia, thrombocytopenia, renal failure, normal coagulation tests and a renal failure, normal coagulation tests and a clear sensorium probably has:clear sensorium probably has:

a.a. idiopathic thrombocytopenic anemia.idiopathic thrombocytopenic anemia.

b.b. thrombotic thrombocytopenic purpura.thrombotic thrombocytopenic purpura.

c.c. hemolytic-uremic syndrome.hemolytic-uremic syndrome.

d.d. disseminated intravascular coagulation. disseminated intravascular coagulation.

e.e. autoimmune hemolytic anemia.autoimmune hemolytic anemia.

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1.1.Hemolytic Uremic SyndromeHemolytic Uremic Syndrome

ITP generally presents with isolated ITP generally presents with isolated thrombocytopenia. TTP causes thrombocytopenia. TTP causes neurologic symptoms in addition to the neurologic symptoms in addition to the other symptoms. DIC will have other symptoms. DIC will have abnormal coagulation studies. abnormal coagulation studies. Autoimmune hemolytic anemia may Autoimmune hemolytic anemia may cause severe rapid anemia, which may cause severe rapid anemia, which may present with angina or congestive heart present with angina or congestive heart failure. failure.

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4.4.Hemolytic-uremic syndrome is most Hemolytic-uremic syndrome is most commonly seen in:commonly seen in:

a.a. neonates.neonates.b.b. infants and children 6 months to 4 infants and children 6 months to 4

years of age .years of age .c.c. adolescents.adolescents.d.d. women age 30 to 50.women age 30 to 50.e.e. both sexes, over age 75.both sexes, over age 75.

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4.4.Hemolytic-uremic syndrome is most Hemolytic-uremic syndrome is most commonly seen in:commonly seen in:

a.a. neonates.neonates.b.b. infants and children 6 months to infants and children 6 months to

4 years of age.4 years of age.c.c. adolescents.adolescents.d.d. women age 30 to 50.women age 30 to 50.e.e. both sexes, over age 75.both sexes, over age 75.

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4.4.Hemolytic-uremic syndrome is most Hemolytic-uremic syndrome is most commonly seen in infants and children.commonly seen in infants and children.

Hemolytic-uremic syndrome (HUS) is a Hemolytic-uremic syndrome (HUS) is a disease mainly of infancy and early disease mainly of infancy and early childhood, with a peak incidence childhood, with a peak incidence between 6 months and 4 years of age.between 6 months and 4 years of age.

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• More common with unfractionatedMore common with unfractionated

• Heparin-dependent antibody Heparin-dependent antibody activates plateletsactivates platelets

• Signs / symptoms occur 5 to 7 days Signs / symptoms occur 5 to 7 days after starting heparinafter starting heparin

• ~50% develop thrombosis~50% develop thrombosis– Venous or arterialVenous or arterial

– Life- and limb-threateningLife- and limb-threatening

Heparin-Induced Heparin-Induced Thrombocytopenia (HIT)Thrombocytopenia (HIT)

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• Moderate thrombocytopenia (~50% Moderate thrombocytopenia (~50% reduction)reduction)

• HIT antibodies in serumHIT antibodies in serum

• Discontinue all heparins, avoid in Discontinue all heparins, avoid in futurefuture

• Avoid warfarin: can progress to Avoid warfarin: can progress to gangrenegangrene

• Other medicines can also causeOther medicines can also cause

Diagnosis & TreatmentDiagnosis & Treatment

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11.11.Heparin-induced thrombocytopenia:Heparin-induced thrombocytopenia:

a.a. does not occur with low molecular does not occur with low molecular weight heparins.weight heparins.

b.b. requires a minimum number of units, requires a minimum number of units, so a heparin so a heparin ““flushflush”” is always safe. is always safe.

c.c. can paradoxically cause thrombosis, can paradoxically cause thrombosis, ischemia, and amputation.ischemia, and amputation.

d.d. never occurs during the first 24 hours never occurs during the first 24 hours of infusion.of infusion.

e.e. is easily treated with warfarin and is easily treated with warfarin and fresh-frozen plasma.fresh-frozen plasma.

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11.11.Heparin-induced thrombocytopenia:Heparin-induced thrombocytopenia:

a.a. does not occur with low molecular does not occur with low molecular weight heparins.weight heparins.

b.b. requires a minimum number of units, requires a minimum number of units, so a heparin so a heparin ““flushflush”” is always safe. is always safe.

c.c. can paradoxically cause can paradoxically cause thrombosis, ischemia, and thrombosis, ischemia, and amputation.amputation.

d.d. never occurs during the first 24 hours never occurs during the first 24 hours of infusion.of infusion.

e.e. is easily treated with warfarin and is easily treated with warfarin and fresh-frozen plasma.fresh-frozen plasma.

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11.11.HIT thrombosisHIT thrombosis

Heparin-induced thrombocytopenia Heparin-induced thrombocytopenia (HIT) is due to an antibody, usually IgG, (HIT) is due to an antibody, usually IgG, which attaches to and stimulates which attaches to and stimulates platelets. This platelet activation platelets. This platelet activation produces both thrombocytopenia and a produces both thrombocytopenia and a tendency for thrombosis. The tendency for thrombosis. The incidence of HIT is between 1 and 3% incidence of HIT is between 1 and 3% in patients treated with unfractionated in patients treated with unfractionated heparin but significantly less in patients heparin but significantly less in patients treated with LMW products.treated with LMW products.

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11.11.HIT thrombosisHIT thrombosis

The onset of HIT is usually 5 to 12 days The onset of HIT is usually 5 to 12 days after heparin treatment is started but after heparin treatment is started but may be sooner for patients who may be sooner for patients who developed the antibody from a previous developed the antibody from a previous exposure. Thrombosis may involve the exposure. Thrombosis may involve the skin (similar to warfarin-induced skin (similar to warfarin-induced cutaneous necrosis), the arteries (e.g., cutaneous necrosis), the arteries (e.g., femoral artery thrombosis), or the veins femoral artery thrombosis), or the veins (e.g., recurrent DVT or PE). (e.g., recurrent DVT or PE).

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• Acute in children (age 2 – 6)Acute in children (age 2 – 6)– Viral prodromeViral prodrome

– Self-limited: remission in 90%Self-limited: remission in 90%

– Treatment: supportiveTreatment: supportive

• Chronic form: adult femalesChronic form: adult females

Idiopathic (Autoimmune) Idiopathic (Autoimmune) Thrombocytopenic Purpura (ITP)Thrombocytopenic Purpura (ITP)

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• Easy bruising, prolonged menses, Easy bruising, prolonged menses, mucosal bleedingmucosal bleeding

• Petechiae, purpuraPetechiae, purpura

• Diagnosis: thrombocytopenia on Diagnosis: thrombocytopenia on CBCCBC

Signs and SymptomsSigns and Symptoms

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• Steroids (?)Steroids (?)

• If refractory: splenectomy or If refractory: splenectomy or immunosuppressive therapyimmunosuppressive therapy

• Platelet transfusion ONLY if serious Platelet transfusion ONLY if serious bleedingbleeding

• Intravenous immunoglobulin G Intravenous immunoglobulin G (IVIG) in children with intracranial (IVIG) in children with intracranial hemorrhage (rare)hemorrhage (rare)

TreatmentTreatment

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14.14.A 5-year-old girl complains of weakness A 5-year-old girl complains of weakness and fatigue. Two weeks ago she saw her and fatigue. Two weeks ago she saw her family doctor and was diagnosed with an family doctor and was diagnosed with an upper respiratory infection. She received no upper respiratory infection. She received no medicine at that time. Physical examination medicine at that time. Physical examination shows only a scattered petechial rash shows only a scattered petechial rash located in areas where her clothing is snug located in areas where her clothing is snug against her skin, such as underwear elastic against her skin, such as underwear elastic lines. Laboratory studies show a white lines. Laboratory studies show a white blood cell count 11,000/mmblood cell count 11,000/mm33 , Hgb 10.5 , Hgb 10.5 mg/dL, and platelet count of 16,000/mmmg/dL, and platelet count of 16,000/mm33. . Appropriate management of this patient Appropriate management of this patient should be: should be: 93

14.14. Recent URI … petechial rash … Recent URI … petechial rash … platelet count of 16,000/mmplatelet count of 16,000/mm3.3.

a.a. platelet concentrate transfusion.platelet concentrate transfusion.b.b. discharge home with instructions to discharge home with instructions to

limit contact sports.limit contact sports.c.c. admit for salicylate therapy.admit for salicylate therapy.d.d. admit for splenectomy.admit for splenectomy.e.e. admit for observation.admit for observation.

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a.a. platelet concentrate transfusion.platelet concentrate transfusion.b.b. discharge home with instructions to discharge home with instructions to

limit contact sports.limit contact sports.c.c. admit for salicylate therapy.admit for salicylate therapy.d.d. admit for splenectomy.admit for splenectomy.e.e. admit for observation.admit for observation.

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Acute idiopathic thrombocytopenic Acute idiopathic thrombocytopenic purpura (ITP) is seen most often in purpura (ITP) is seen most often in children 2 to 6 years old. A viral children 2 to 6 years old. A viral prodrome is common, usually within 3 prodrome is common, usually within 3 weeks of the onset. The platelet count weeks of the onset. The platelet count falls, usually to <20,000/mmfalls, usually to <20,000/mm33. The . The course is self-limited, with a greater course is self-limited, with a greater than 90% rate of spontaneous than 90% rate of spontaneous remission. Morbidity and mortality are remission. Morbidity and mortality are low. Treatment is supportive. Steroid low. Treatment is supportive. Steroid therapy does not alter disease course. therapy does not alter disease course.

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• Lymphadenopathy: mostly above Lymphadenopathy: mostly above diaphragm (lower neck, diaphragm (lower neck, subclavicular)subclavicular)

• Large mediastinal mass Large mediastinal mass chest chest pressure, cough, dyspneapressure, cough, dyspnea

• B symptoms: weight loss, fever, B symptoms: weight loss, fever, night sweatsnight sweats– May be presenting complaintMay be presenting complaint

Lymphoma: HodgkinLymphoma: Hodgkin

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• Diagnosis: lymph node biopsy Diagnosis: lymph node biopsy showing Reed-Sternberg cellsshowing Reed-Sternberg cells

• Treatment: chemotherapy / Treatment: chemotherapy / radiationradiation

Lymphoma: HodgkinLymphoma: Hodgkin

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• Proliferation of lymphoid cells from Proliferation of lymphoid cells from lymph nodes OR lymphatic tissue lymph nodes OR lymphatic tissue other than bone marrowother than bone marrow

• B-Cell, T-Cell, NK-Cell: can be B-Cell, T-Cell, NK-Cell: can be indolent or aggressiveindolent or aggressive

• Diagnosis: biopsyDiagnosis: biopsy

• Treatment: chemotherapy / Treatment: chemotherapy / radiationradiation

Non-Hodgkin LymphomaNon-Hodgkin Lymphoma

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• Anemia + thrombocytopenia + Anemia + thrombocytopenia + neutropenianeutropenia

• Bone marrow diseases: myelo-Bone marrow diseases: myelo-dysplasia, myelofibrosis, some dysplasia, myelofibrosis, some leukemias and lymphomasleukemias and lymphomas

• Systemic diseases: lupus, severe Systemic diseases: lupus, severe infection, sarcoidosis, alcohol, infection, sarcoidosis, alcohol, tuberculosis, tuberculosis, vitamin Bvitamin B1212, folate, folate

PancytopeniaPancytopenia

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• Pancytopenia with hypocellular Pancytopenia with hypocellular bone marrowbone marrow– Acquired: marrow stem cells Acquired: marrow stem cells

damaged by drugs, radiation, virus, damaged by drugs, radiation, virus, chemical, immune-relatedchemical, immune-related

– Inherited: FanconiInherited: Fanconi’’s anemia, s anemia, dyskeratosis congenitadyskeratosis congenita

Aplastic AnemiaAplastic Anemia

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• Bleeding, easy bruisingBleeding, easy bruising

• Fatigue, malaise, anemia Fatigue, malaise, anemia short short of breathof breath

• Infection commonInfection common

Signs & SymptomsSigns & Symptoms

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• CBC, reticulocyte countCBC, reticulocyte count

• Bone marrow biopsyBone marrow biopsy

• Immediate: depends on severity of Immediate: depends on severity of anemia / thrombocytopenia / anemia / thrombocytopenia / neutropenia ± complicationsneutropenia ± complications

• Bone marrow transplantBone marrow transplant

Diagnosis / TreatmentDiagnosis / Treatment

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Red Blood Cells (RBC)Red Blood Cells (RBC)

• Normal life: ~120 daysNormal life: ~120 days

• Marrow releases ~1% total count Marrow releases ~1% total count dailydaily– ““baby red cellsbaby red cells”” = nucleated = = nucleated =

reticulocytesreticulocytes

106

7.7.The most helpful laboratory study to The most helpful laboratory study to differentiate poor red blood cell production differentiate poor red blood cell production from increased red cell destruction is the:from increased red cell destruction is the:

a.a. sedimentation rate.sedimentation rate.b.b. sideroblast level.sideroblast level.c.c. serum iron level.serum iron level.d.d. total to direct bilirubin ratio.total to direct bilirubin ratio.e.e. reticulocyte count.reticulocyte count.

107

7.7.The most helpful laboratory study to The most helpful laboratory study to differentiate poor red blood cell production differentiate poor red blood cell production from increased red cell destruction is the:from increased red cell destruction is the:

a.a. sedimentation rate.sedimentation rate.b.b. sideroblast level.sideroblast level.c.c. serum iron level.serum iron level.d.d. total to direct bilirubin ratio.total to direct bilirubin ratio.e.e. reticulocyte count.reticulocyte count.

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7.7. production vs production vs destruction destruction

Reticulocytes are RBCs of intermediate Reticulocytes are RBCs of intermediate maturity. They are an index of the maturity. They are an index of the production of mature RBCs by the bone production of mature RBCs by the bone marrow (reported as a percent of total marrow (reported as a percent of total RBCs). RBCs). reticulocyte count reflects reticulocyte count reflects impaired RBC production; seen with impaired RBC production; seen with low levels of iron, vitamin B12, folate, low levels of iron, vitamin B12, folate, bone marrow failure. bone marrow failure. reticulocyte reticulocyte count reflects accelerated count reflects accelerated erythropoeisis, the normal marrow erythropoeisis, the normal marrow response to anemia; seen with blood response to anemia; seen with blood loss and hemolytic anemias. loss and hemolytic anemias. 109

Aplastic AnemiaAplastic Anemia

• Normal MCV with Normal MCV with reticulocyte reticulocyte

• Can be RBC aplasia aloneCan be RBC aplasia alone

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Sickle Cell DiseaseSickle Cell Disease

• Inherited mutated hemoglobin: HbSInherited mutated hemoglobin: HbS

• Recessive gene: both parents need Recessive gene: both parents need to pass to offspringto pass to offspring

• HbS + HbA: trait HbS + HbA: trait asymptomatic asymptomatic

• HbS + HbC (beta-thalassemia): HbS + HbC (beta-thalassemia): chronic anemia + recurrent painful chronic anemia + recurrent painful crisescrises

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Sickle Cell DiseaseSickle Cell Disease

• RBC life span 10 – 15 daysRBC life span 10 – 15 days

• Marrow on constant Marrow on constant ““overdriveoverdrive””

• Reticulocyte Reticulocyte ““normalnormal”” 8 – 10% 8 – 10%

• WBC WBC ““normalnormal”” at 15 – 20K at 15 – 20K

• Hemoglobin Hemoglobin ““normalnormal”” at 8 – 10 at 8 – 10

• Platelets Platelets ““normalnormal”” >500K >500K

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Vaso-Occlusive CrisisVaso-Occlusive Crisis

• AKA acute painful episodeAKA acute painful episode

• Pain in extremities, chest, Pain in extremities, chest, abdomen, backabdomen, back

• No lab test can confirmNo lab test can confirm

• No vital sign can confirmNo vital sign can confirm

• Treatment: analgesiaTreatment: analgesia– Avoid meperidineAvoid meperidine

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Acute Chest SyndromeAcute Chest Syndrome

• New infiltrate on chest x-ray + New infiltrate on chest x-ray + fever, cough, fever, cough, sputum, dyspnea, sputum, dyspnea, tachypnea, hypoxiatachypnea, hypoxia

• Many causes: fat embolism from Many causes: fat embolism from marrow ischemia, pulmonary vaso-marrow ischemia, pulmonary vaso-occlusion, infection, venous occlusion, infection, venous thromboembolism, pulmonary thromboembolism, pulmonary edemaedema

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• Children: fever & cough most Children: fever & cough most commoncommon

• Adults: fever & chest pain as Adults: fever & chest pain as presenting complaintpresenting complaint– Some develop 2 – 3 days into Some develop 2 – 3 days into

hospital stayhospital stay

• ± hypoxemia± hypoxemia

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• Chest x-ray normal in ~50%Chest x-ray normal in ~50%

• Treatment supportiveTreatment supportive

• Empiric antibiotic: 3Empiric antibiotic: 3rdrd / 4 / 4thth generation cephalosporin + generation cephalosporin + macrolidemacrolide

• Severe Severe exchange transfusion exchange transfusion

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Splenic SequestrationSplenic Sequestration

• Etiology: unknownEtiology: unknown

• Typically occurs in infantsTypically occurs in infants– Tender, enlarged spleenTender, enlarged spleen

– Signs of hypovolemia, shockSigns of hypovolemia, shock

– Concurrent infection commonConcurrent infection common

• Diagnosis: worsening anemia, Diagnosis: worsening anemia, persistent reticulocytisis, tender persistent reticulocytisis, tender large spleenlarge spleen

117

Splenic SequestrationSplenic Sequestration

• Treatment:Treatment:– Intravenous fluidsIntravenous fluids

– Blood transfusionBlood transfusion

– May need splenectomy after acute May need splenectomy after acute episode resolvesepisode resolves

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Aplastic CrisisAplastic Crisis

• Marrow shuts downMarrow shuts down

• Abrupt Abrupt hemoglobin hemoglobin

• Abrupt Abrupt reticulocytes (<2%) reticulocytes (<2%)

• Causes: folate deficiency, human Causes: folate deficiency, human parvovirus B19 (in children)parvovirus B19 (in children)

• Blood transfusion: severe anemia, Blood transfusion: severe anemia, cardiorespiratory symptomscardiorespiratory symptoms

119

Hemolytic CrisisHemolytic Crisis

• Abrupt Abrupt hemoglobin hemoglobin

• Major increase reticulocyte Major increase reticulocyte productionproduction

• May see worsening jaundiceMay see worsening jaundice

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Other ComplicationsOther Complications

• Functional asplenia: infected by Functional asplenia: infected by encapsulated organismsencapsulated organisms

• Strokes: CNS occlusive eventsStrokes: CNS occlusive events

• Priapism: low-flow due to corpora Priapism: low-flow due to corpora cavernosa occlusioncavernosa occlusion

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2.2.The viral agent implicated in an aplastic The viral agent implicated in an aplastic crisis of patients with sickle cell disease is:crisis of patients with sickle cell disease is:

a.a. adenovirus (atypical).adenovirus (atypical).

b.b. herpes simplex.herpes simplex.

c.c. parvovirus.parvovirus.

d.d. coxsackie virus.coxsackie virus.

e.e. HTLV-IV.HTLV-IV.

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2.2.The viral agent implicated in an aplastic The viral agent implicated in an aplastic crisis of patients with sickle cell disease is:crisis of patients with sickle cell disease is:

a.a. adenovirus (atypical).adenovirus (atypical).

b.b. herpes simplex.herpes simplex.

c.c. parvovirus.parvovirus.

d.d. coxsackie virus.coxsackie virus.

e.e. HTLV-IV.HTLV-IV.

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2.2.Parvovirus & Sickle cellParvovirus & Sickle cell

Aplastic crises can be precipitated by Aplastic crises can be precipitated by viral infections (particularly parvovirus viral infections (particularly parvovirus B19), folic acid deficiency, or the B19), folic acid deficiency, or the ingestion of bone marrow toxins such ingestion of bone marrow toxins such as phenylbutazone. Bone marrow as phenylbutazone. Bone marrow erythropoiesis is slowed or stopped. erythropoiesis is slowed or stopped. The hematocrit falls to as low as 10%, The hematocrit falls to as low as 10%, and the reticulocyte count falls to as and the reticulocyte count falls to as low as 0.5%. The white blood cell low as 0.5%. The white blood cell count and platelet counts usually count and platelet counts usually remain stable. remain stable. 124

9.9.A 12-year-old girl with sickle cell disease is A 12-year-old girl with sickle cell disease is brought by her mother after she passed out brought by her mother after she passed out twice. She was kept home from school the twice. She was kept home from school the last few days for a last few days for a ““cold.cold.”” When you ask the When you ask the child to stand, you must catch her to prevent child to stand, you must catch her to prevent her from falling to the ground. This is her from falling to the ground. This is suspicious for:suspicious for:a.a. salmonella sepsis.salmonella sepsis.b.b. sequestration crisis.sequestration crisis.c.c. acute chest syndrome.acute chest syndrome.d.d. aplastic crisis.aplastic crisis.e.e. hemolytic crisis.hemolytic crisis.

125

9.9.A 12-year-old girl with sickle cell disease is A 12-year-old girl with sickle cell disease is brought by her mother after she passed out brought by her mother after she passed out twice. She was kept home from school the twice. She was kept home from school the last few days for a last few days for a ““cold.cold.”” When you ask the When you ask the child to stand, you must catch her to prevent child to stand, you must catch her to prevent her from falling to the ground. This is her from falling to the ground. This is suspicious for:suspicious for:a.a. salmonella sepsis.salmonella sepsis.b.b. sequestration crisis.sequestration crisis.c.c. acute chest syndrome.acute chest syndrome.d.d. aplastic crisis.aplastic crisis.e.e. hemolytic crisis.hemolytic crisis.

126

9.9.Sequestration crisisSequestration crisis

Sequestration crisis occurs primarily in Sequestration crisis occurs primarily in children and is the second most children and is the second most common cause of death in children with common cause of death in children with SCD under the age of 5 years. Often SCD under the age of 5 years. Often preceded by viral infections, sickled preceded by viral infections, sickled cells block the splenic outflow, causing cells block the splenic outflow, causing pooling of peripheral blood and sickled pooling of peripheral blood and sickled cells in the spleen. Such patients cells in the spleen. Such patients present in hypovolemic shock with an present in hypovolemic shock with an enlarged spleen. enlarged spleen.

127

Hemolytic AnemiaHemolytic Anemia

• RBC destructionRBC destruction

• IntravascularIntravascular– Smear Smear fragmented RBCs fragmented RBCs

(schistocytes)(schistocytes)

– More acute than extravascularMore acute than extravascular

• ExtravascularExtravascular– Occurs in liver or spleenOccurs in liver or spleen

– Smear Smear spherocytes spherocytes128


• IntrinsicIntrinsic– Enzyme defect (G6PD, pyruvate Enzyme defect (G6PD, pyruvate

kinase), abnormal membrane, kinase), abnormal membrane, abnormal cell (sickle cell disease, abnormal cell (sickle cell disease, thalassemia)thalassemia)

– G6PD triggered by drugs (aspirin, G6PD triggered by drugs (aspirin, antimalarials, sulfa, nitrofurantoin, antimalarials, sulfa, nitrofurantoin, phenazopyridine), foods (fava beans), phenazopyridine), foods (fava beans), infectioninfection

129


• ExtrinsicExtrinsic– AutoimmuneAutoimmune

– Non-immune: HUS/TTP, HELLP, Non-immune: HUS/TTP, HELLP, prosthetic valve abnormality, arterio-prosthetic valve abnormality, arterio-venous malformation, malaria, drugs, venous malformation, malaria, drugs, spider /snake venomspider /snake venom

130


• Vary depending on diseaseVary depending on disease

• Symptoms related to anemiaSymptoms related to anemia

• JaundiceJaundice

• Hepatosplenomegaly Hepatosplenomegaly

131

DiagnosisDiagnosis

• Smear Smear schisto-, spherocytes schisto-, spherocytes

• Reticulocytes should be Reticulocytes should be • Haptoglobin: binds free HgbHaptoglobin: binds free Hgb • Bilirubin Bilirubin • LDH LDH (released from RBCs) (released from RBCs)

• Hemoglobinemia, hemoglobinuriaHemoglobinemia, hemoglobinuria

• Autoimmune Autoimmune direct Coombs + direct Coombs +132

Hypochromic Microcytic AnemiaHypochromic Microcytic Anemia

iron iron iron deficiency iron deficiency globin globin thalassemia thalassemia porphyrin porphyrin sideroblastic, lead sideroblastic, lead

toxicitytoxicity• Chronic diseaseChronic disease

133

13.13. A healthy 12-year-old African-A healthy 12-year-old African-American female complains of American female complains of weakness and fatigue 3 days after weakness and fatigue 3 days after starting a course of trimethoprim-starting a course of trimethoprim-sulfamethoxasole and pyridium for sulfamethoxasole and pyridium for a urinary tract infection. Her a urinary tract infection. Her hemoglobin is 4.8 mg/dl, and her hemoglobin is 4.8 mg/dl, and her urine is tea-colored, but you see no urine is tea-colored, but you see no red blood cells on microscopic red blood cells on microscopic exam. She probably has exam. She probably has undiagnosed:undiagnosed:

134

13.13.12-year-old African-American 12-year-old African-American female…weakness and fatigue… female…weakness and fatigue… TMP/SMZ … hgb 4.8 mg/dl … no TMP/SMZ … hgb 4.8 mg/dl … no RBCs in urine. Undiagnosed:RBCs in urine. Undiagnosed:a.a. hemolytic uremic syndrome.hemolytic uremic syndrome.b.b. G6PD deficiency.G6PD deficiency.c.c. idiopathic thrombocytopenic purpura.idiopathic thrombocytopenic purpura.d.d. thrombotic thrombocytopenic thrombotic thrombocytopenic

purpura.purpura.e.e. sickle cell disease.sickle cell disease.

135

13.13.12-year-old African-American 12-year-old African-American female…weakness and fatigue… female…weakness and fatigue… TMP/SMZ … hgb 4.8 mg/dl … no TMP/SMZ … hgb 4.8 mg/dl … no RBCs in urine. Undiagnosed:RBCs in urine. Undiagnosed:a.a. hemolytic uremic syndrome.hemolytic uremic syndrome.b.b. G6PD deficiency.G6PD deficiency.c.c. idiopathic thrombocytopenic purpura.idiopathic thrombocytopenic purpura.d.d. thrombotic thrombocytopenic thrombotic thrombocytopenic

purpura.purpura.e.e. sickle cell disease.sickle cell disease.

136

13.13.G6PDG6PD

Deficiency of the RBC enzyme Deficiency of the RBC enzyme glucose-6-phosphate dehydrogenase glucose-6-phosphate dehydrogenase (G-6-PD) is the most common human (G-6-PD) is the most common human enzyme defect, affecting nearly one-enzyme defect, affecting nearly one-tenth of the worldtenth of the world’’s population. The s population. The RBC is unable to protect itself against RBC is unable to protect itself against oxidant stress. Acute hemolytic crises oxidant stress. Acute hemolytic crises occur that are incited by bacterial and occur that are incited by bacterial and viral infections, exposure to oxidant viral infections, exposure to oxidant drugs, metabolic acidosis (such as drugs, metabolic acidosis (such as diabetic ketoacidosis), and ingestion of diabetic ketoacidosis), and ingestion of fava beans in some patients.fava beans in some patients.

137

13.13.G6PDG6PD

Within 1 to 3 days following oxidant Within 1 to 3 days following oxidant stress, the patient can develop stress, the patient can develop hemoglobinuria and the potential for hemoglobinuria and the potential for vascular collapse. These hemolytic vascular collapse. These hemolytic crises are generally well tolerated and crises are generally well tolerated and self-limited because only the older self-limited because only the older RBCs will hemolyze. The drugs most RBCs will hemolyze. The drugs most commonly associated with oxidant commonly associated with oxidant stress are sulfa drugs, antimalarials, stress are sulfa drugs, antimalarials, phenazopyridine, and nitrofurantoin. phenazopyridine, and nitrofurantoin.

138


serum iron levelserum iron level serum ferritin levelserum ferritin level total iron binding capacity total iron binding capacity

(TIBC)(TIBC)• Treatment: iron supplements, Treatment: iron supplements,

outpatient workupoutpatient workup

139

Macrocytic AnemiaMacrocytic Anemia

• Most important: megaloblasticMost important: megaloblastic folate (green vegetables, fruit, folate (green vegetables, fruit,

cereals) or cereals) or vitamin B12 (meat) vitamin B12 (meat) impaired DNA synthesis impaired DNA synthesis

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Macrocytic AnemiaMacrocytic Anemia

• Petechiae, mucosal bleeding, Petechiae, mucosal bleeding, infections, sore mouth / tongue, infections, sore mouth / tongue, diarrhea, weight lossdiarrhea, weight loss

• BB1212 can also have paresthesias, can also have paresthesias,

ataxiaataxia• Diagnosis: send levelsDiagnosis: send levels• Treatment: replaceTreatment: replace

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PolycythemiaPolycythemia

Hct: >51% in M, >48% in FHct: >51% in M, >48% in F• Apparent: Apparent: plasma volume plasma volume• SecondarySecondary

– Appropriate: hypoxiaAppropriate: hypoxia– Inappropriate: Inappropriate: erythropoietin erythropoietin

• Polycythemia vera: Polycythemia vera: production production RBCs, WBCs, plateletsRBCs, WBCs, platelets– Can progress to myelofibrosis, Can progress to myelofibrosis,

leukemialeukemia142


• Headache, weakness, pruritus, Headache, weakness, pruritus, dizziness, dizziness, sweating, visual sweating, visual changes, paresthesias, weight loss, changes, paresthesias, weight loss, joint painjoint pain

• Ruddy complexion, Ruddy complexion, spleen, spleen, liver, liver, blood pressureblood pressure

• Thrombosis: ACS, CVA, PE, etcThrombosis: ACS, CVA, PE, etc• Hemorrhage Hemorrhage

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RBCs, RBCs, WBCs, WBCs, plateletsplatelets• Confirmed by non-ED studiesConfirmed by non-ED studies• Treatment: phlebotomyTreatment: phlebotomy

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16.16. A 68-year-old man complains of A 68-year-old man complains of headache, dizziness, and blurred headache, dizziness, and blurred vision. His blood pressure is vision. His blood pressure is 190/118 mmHg. He has a florid 190/118 mmHg. He has a florid face, normal fundi, and marked face, normal fundi, and marked splenomegaly. His hematocrit is splenomegaly. His hematocrit is 67%. Reasonable therapy 67%. Reasonable therapy includes:includes:

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16.16. … … florid face … splenomegaly … florid face … splenomegaly … hematocrit = 67%. Therapy:hematocrit = 67%. Therapy:

a.a. 250cc salt-poor albumin.250cc salt-poor albumin.b.b. intravenous nitroprusside.intravenous nitroprusside.c.c. phlebotomy.phlebotomy.d.d. plasmapheresis.plasmapheresis.e.e. sublingual nifedipine.sublingual nifedipine.

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a.a. 250cc salt-poor albumin.250cc salt-poor albumin.b.b. intravenous nitroprusside.intravenous nitroprusside.c.c. phlebotomy.phlebotomy.d.d. plasmapheresis.plasmapheresis.e.e. sublingual nifedipine.sublingual nifedipine.

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Emergency treatment of any form of Emergency treatment of any form of symptomatic polycythemia is symptomatic polycythemia is phlebotomy. Usually not more than phlebotomy. Usually not more than 500 ml of blood is slowly removed as 500 ml of blood is slowly removed as the volume is replaced with a the volume is replaced with a comparable amount of normal saline. comparable amount of normal saline.

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MethemoglobinemiaMethemoglobinemia

production or production or reductionreduction• Causes discussed in toxicologyCauses discussed in toxicology• Cyanosis with normal oxygen Cyanosis with normal oxygen

saturationsaturation• Treatment:Treatment:

– Congenital: usually noneCongenital: usually none– Toxic Toxic symptomatic symptomatic treat with treat with

intravenous methylene blueintravenous methylene blue

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LeukemiaLeukemia

production undifferentiated production undifferentiated hematopoietic stem cellshematopoietic stem cells

• Acute Lymphocytic (ALL)Acute Lymphocytic (ALL)• Signs & symptoms: proteanSigns & symptoms: protean

– Related to abnormal cells crowding Related to abnormal cells crowding out normal cells out normal cells RBC, RBC, WBC, WBC, plateletsplatelets

– Bacterial infections in 1/3 at time of Bacterial infections in 1/3 at time of diagnosisdiagnosis

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LeukemiaLeukemia

• Chronic Lymphocytic (CLL): most Chronic Lymphocytic (CLL): most common in patients >50 yearscommon in patients >50 years

• Often no symptoms: may have Often no symptoms: may have fatigue, large lymph nodes, fatigue, large lymph nodes, infections (esp. respiratory)infections (esp. respiratory)

• Diagnosis: absolute lymphocyte Diagnosis: absolute lymphocyte count >5000 cells/mlcount >5000 cells/ml

• Treatment: monitor, chemoTreatment: monitor, chemo

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Multiple MyelomaMultiple Myeloma

• Abnormal Abnormal plasma cellsplasma cells• Fatigue from anemiaFatigue from anemia• Bone pain from osteolytic lesions or Bone pain from osteolytic lesions or

pathologic fracturespathologic fractures• Diagnosis: Diagnosis: calcium, calcium, creatininecreatinine• Bone marrow: >10% plasma cellsBone marrow: >10% plasma cells• Serum / urine protein electro-Serum / urine protein electro-

phoresis (SPEP, UPEP)phoresis (SPEP, UPEP)

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TreatmentTreatment

• ChemotherapyChemotherapy• Plasmapheresis for hyperviscosity Plasmapheresis for hyperviscosity

syndromesyndrome• If comatose: temporize by removing If comatose: temporize by removing

1 liter blood, replace with normal 1 liter blood, replace with normal salinesaline

calcium: IV saline, steroidscalcium: IV saline, steroids

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Malignancy ComplicationsMalignancy Complications

• Hyperviscosity syndromeHyperviscosity syndrome• Leukostasis (sludging)Leukostasis (sludging)• Neutropenic feverNeutropenic fever• Spinal cord compressionsSpinal cord compressions• Superior vena cava syndromeSuperior vena cava syndrome• Tumor lysis syndromeTumor lysis syndrome• HypercalcemiaHypercalcemia• Pericardial effusion / tamponadePericardial effusion / tamponade

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Hyperviscosity SyndromeHyperviscosity Syndrome

abnormal serum proteinsabnormal serum proteins• Waldenstrom macroglobulinemiaWaldenstrom macroglobulinemia• Multiple myeloma (less common)Multiple myeloma (less common)• Most common symptoms: Most common symptoms:

neurologic, visualneurologic, visual• May see mucosal or GI bleedingMay see mucosal or GI bleeding• CHF from CHF from plasma volume plasma volume• Plasmapheresis, exchangePlasmapheresis, exchange

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LeukostasisLeukostasis

• WBC sludging in microcirculationWBC sludging in microcirculation• Usually acute leukemiaUsually acute leukemia• Can be seen with chronic, NHLCan be seen with chronic, NHL• Neurologic symptomsNeurologic symptoms• Can see respiratory failureCan see respiratory failure• Treatment: leukapheresis, Treatment: leukapheresis,

hydroxyurea, chemotherapyhydroxyurea, chemotherapy

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Neutropenic FeverNeutropenic Fever

• Absolute neutrophil count (ANC) = Absolute neutrophil count (ANC) = neutrophils + bandsneutrophils + bands

• ANC <500 cells/mlANC <500 cells/ml• Signs and symptoms: feverSigns and symptoms: fever• Treatment: IV antibioticsTreatment: IV antibiotics

– Ceftazidime or cefepime ± amino-Ceftazidime or cefepime ± amino-glycoside or –penemglycoside or –penem

– Add vancomycin if appropriateAdd vancomycin if appropriate

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Spinal Cord CompressionSpinal Cord Compression

• Lymphoma, metastasis, primaryLymphoma, metastasis, primary• S&S: back pain, weakness, S&S: back pain, weakness,

numbness, bowel / bladder / sexual numbness, bowel / bladder / sexual dysfunctiondysfunction

• Diagnosis: MRIDiagnosis: MRI– CT myelogram if MRI not availableCT myelogram if MRI not available

• TreatmentTreatment– DexamethasoneDexamethasone– Radiation therapyRadiation therapy

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Superior Vena Cava SyndromeSuperior Vena Cava Syndrome

• Tumor obstructs SVC Tumor obstructs SVC venous venous hypertension, congestionhypertension, congestion

• Early signs: face edema that Early signs: face edema that improves through dayimproves through day

• SOB, cough, chest painSOB, cough, chest pain• Distension of chest, neck veinsDistension of chest, neck veins• CyanosisCyanosis• Diagnosis: chest CTDiagnosis: chest CT• Treatment: radiation therapyTreatment: radiation therapy

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Acute Tumor Lysis SyndromeAcute Tumor Lysis Syndrome

• Usually after chemotherapyUsually after chemotherapy• Lysed cells Lysed cells metabolic changes metabolic changes• Hyperuricemia: N/V, renal failure Hyperuricemia: N/V, renal failure

due to renal precipitationdue to renal precipitation• Hyperphosphatemia: N/V, lethargy, Hyperphosphatemia: N/V, lethargy,

seizures, renal impairseizures, renal impair– Also binds with CaAlso binds with Ca++ calciumcalcium

• Hypocalcemia: tetany, arrhythmiaHypocalcemia: tetany, arrhythmia

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Acute Tumor Lysis SyndromeAcute Tumor Lysis Syndrome

• Hyperkalemia: N/V, muscle Hyperkalemia: N/V, muscle weakness, cramps, arrhythmias, weakness, cramps, arrhythmias, heart block heart block asystole asystole

• Acute renal failure: possible Acute renal failure: possible hemodialysis; do NOT alkalinize hemodialysis; do NOT alkalinize urine, worsens urine, worsens phosphorus and phosphorus and calciumcalcium

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OthersOthers

• Hypercalcemia: see Endocrine-Hypercalcemia: see Endocrine-Metabolic EmergenciesMetabolic Emergencies

• Pericardial Effusion / Tamponade: Pericardial Effusion / Tamponade: see Cardiovascular Emergenciessee Cardiovascular Emergencies

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12.12.A 42-year-old woman with adult T-cell A 42-year-old woman with adult T-cell lymphoma-leukemia complains of back pain, lymphoma-leukemia complains of back pain, abdominal pain, and confusion. Laboratory abdominal pain, and confusion. Laboratory evaluation shows a total calcium of 15.8 evaluation shows a total calcium of 15.8 mg/dl. Appropriate management of this mg/dl. Appropriate management of this patient should include:patient should include:

a.a. plasmapheresis.plasmapheresis.b.b. IV bicarbonate.IV bicarbonate.c.c. IV hypertonic saline / oral IV hypertonic saline / oral

Kayexalate®.Kayexalate®.d.d. IV normal saline and IV furosemide.IV normal saline and IV furosemide.e.e. glucagon.glucagon.

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12.12.A 42-year-old woman with adult T-cell A 42-year-old woman with adult T-cell lymphoma-leukemia complains of back pain, lymphoma-leukemia complains of back pain, abdominal pain, and confusion. Laboratory abdominal pain, and confusion. Laboratory evaluation shows a total calcium of 15.8 evaluation shows a total calcium of 15.8 mg/dl. Appropriate management of this mg/dl. Appropriate management of this patient should include:patient should include:a.a. plasmapheresis.plasmapheresis.b.b. IV bicarbonate.IV bicarbonate.c.c. IV hypertonic saline / oral Kayexalate®.IV hypertonic saline / oral Kayexalate®.

d.d. IV normal saline and IV IV normal saline and IV furosemide.furosemide.

e.e. glucagon.glucagon.

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12.12.Hypercalcemia of malignancyHypercalcemia of malignancy

Patients with severe hypercalcemia Patients with severe hypercalcemia (>14 mg/dl) require immediate (>14 mg/dl) require immediate treatment regardless of symptoms. treatment regardless of symptoms. The four basic goals of therapy areThe four basic goals of therapy are

(1)(1)restore intravascular volume, restore intravascular volume, (2)(2)enhance renal calcium eliminationenhance renal calcium elimination(3)(3)reduce osteoclastic activityreduce osteoclastic activity(4)(4)treat primary disorder.treat primary disorder.

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12.12.Hypercalcemia of malignancyHypercalcemia of malignancy

Isotonic saline is the first step. Once Isotonic saline is the first step. Once volume is restored, the calcium will volume is restored, the calcium will usually have usually have by 1.6 to 2.4 mg/dl, but by 1.6 to 2.4 mg/dl, but hydration alone rarely leads to hydration alone rarely leads to complete normalization. Loop diuretics complete normalization. Loop diuretics inhibit resorption of calcium in the thick inhibit resorption of calcium in the thick ascending loop of Henle, ascending loop of Henle, the the calciuric effect of hydration. Volume calciuric effect of hydration. Volume expansion must precede administration expansion must precede administration of furosemide, because the drugof furosemide, because the drug’’s s effect depends on the delivery of effect depends on the delivery of calcium to the distal nephron. calcium to the distal nephron. 168

PearlsPearls

• The first step in managing any The first step in managing any transfusion reaction to to stop the transfusion reaction to to stop the transfusiontransfusion

• Hemarthrosis in hemophiliacs: Hemarthrosis in hemophiliacs: factor replacement, never factor replacement, never arthrocentesisarthrocentesis

• Minor head injury in hemophilia: Minor head injury in hemophilia: replace factor, head CT, admit for replace factor, head CT, admit for observationobservation

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PearlsPearls

• Initial treatment in mild-moderate Initial treatment in mild-moderate hemophilia A: DDAVPhemophilia A: DDAVP

• NO PLATELET TRANSFUSION in NO PLATELET TRANSFUSION in patients with TTP, HUSpatients with TTP, HUS

• Once a patient has HIT, no heparin Once a patient has HIT, no heparin can ever again be used, including can ever again be used, including LMWHLMWH

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Thank You

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Project: Ghana Emergency Medicine Collaborative, 2013 Document Title: Hematologic and Oncologic...

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Transcript of Project: Ghana Emergency Medicine Collaborative, 2013 Document Title: Hematologic and Oncologic...