HEMATOLOGIC EMERGENCIES

Jason MitchellMichael Szava-Kovats

Joe VipondMay 17, 2012

HEMOSTASIS

HEMOSTASIS

HEMOSTASIS

HEMOSTASIS

HEMOSTASIS

PTT PT/INR

CASE

30 yo M Tooth extraction Consistent oozing Stable

DDx? Investigations?

CASE

135

7 130

140

111

4.0

24

63

PTT: 54

INR: 0.9

von WILLEBRAND DISEASE

von WILLEBRAND DISEASE

THREE TYPES

Type I: Partial quantitative deficiency vWF AD, Most common (75%)

Type II: Abnormally functioning vWF AD or AR, Subtypes: IIA, IIB, IIM, IIN

Type III: Total quantitative deficiency vWF AR, Leads to severe bleeding

von WILLEBRAND DISEASE

INVESTIGATIONS

Plasma vWF:Ag Decreased Plasma vWF Activity Decreased FVIII Levels Decreased

DISEASE PTT PT/INR PLATELET #

vWD N N

von WILLEBRAND DISEASE

MANAGEMENT OPTIONSStandard measures

DDAVP

vWF Replacement

von WILLEBRAND DISEASE

von WILLEBRAND DISEASE

DESMOPRESSIN (DDAVP/OCTOSTIM)

0.3 μg/kg IV (MAX 20 μg) SC/IV over 20-30 minutes

von WILLEBRAND DISEASE

vWF REPLACEMENT

Humate P 60 – 80 Units/kg IV

von WILLEBRAND DISEASE

?r-vWF

Some promising results in animal studies

Currently in phase III human trials.

HEMOSTASIS

CASE

51 yo M Fall down 3 stairs, Stable No LOC, no neuro deficits No neck pain C/O R knee pain/swelling Hemophiliac

HEMOPHILIA

Management priorities?

HEMOPHILIA

HEMOPHILIA A

Factor VIII Deficiency

X-linked recessive

1:5-10 000 males

HEMOPHILIA B

Factor IX Deficiency

X-linked recessive

1:25-30 000 males

HEMOPHILIA

Initial Investigations?

HEMOPHILIA

105

13

115

138

101

4.1

19

76

PTT: 76

INR: 0.9

HEMOPHILIA

INVESTIGATIONS

Factor VIII Level Decreased Factor IX Level Decreased

DISEASE PTT PT/INR PLATELET #

HEMOPHILIA N N

HEMOPHILIA

MANAGEMENT Depends on:

Clotting factor activity level

Location of bleed

HEMOPHILIA

SEVERITY % FACTOR ACTIVITY

Mild > 5

Moderate 1 - 5

Severe < 1

HEMOPHILIA

BLEEDING LOCATION

LIFE/LIMB THREATENING BLEED

MODERATE/MINOR BLEED

HEMOPHILIA

LIFE/LIMB THREATENING BLEED

HEMOPHILIA

MILD/MODERATE BLEED

HEMOPHILIA

MANAGEMENT OPTIONSCryoprecipitateFFPDDAVPrFVIII / rFIXAntifibrinolytics

HEMOPHILIA

CRYOPRECIPITATE

FVIII, vWF, Fibrinogen

2 bags / 10 kg

Not first line therapy – use if rFVIII not available

FFP

All coagulation factors

Not ideal

Need to double plasma volume level

HEMOPHILIA

DESMOPRESSIN (DDAVP/OCTOSTIM)

0.3 μg/kg IV (MAX 20 μg) SC/IV over 20-30 minutes

HEMOPHILIA rFVIII (KOGENATE)

MILD/MODERATE 30 U/kg

SEVERE 50 U/kg

rFIX (BeneFIX) MILD/MODERATE

50 U/kg > 15 70 U/kg < 15

SEVERE 120 U/kg > 15 160 U/kg < 15

HEMOPHILIA

WHEN IN DOUBT, TREAT AS SEVERE BLEED!

HEMOPHILIA

ANTIFIBRINOLYTICS

Tranexamic Acid

Epsilon Aminocaproic Acid

HEMOPHILIA

PLASMINOGEN

HEMOPHILIA

ANTIFIBRINOLYTICS

25 mg/kg PO TID 1-7 days

HEMOPHILIA

THERAPEUTIC ENDPOINTS

Bleeding cessation

Symptom resolution

Correction of PTT

Raised factor activity level

BACK TO CASE

51 yo M Fell down three stairs Stable R knee hemarthrosis

HEMOPHILIA A

HEMOPHILIA

What type of bleed?

HEMOPHILIA A

HEMOPHILIA A

Management?

HEMOPHILIA

HEMOPHILIA

Why is the patient not responding?

HEMOPHILIA

INHIBITORS

Formation of anti-factor antibodies (IgG)

1/3 severe Hemophilia A; 1/50 mild/moderate Hemophelia A

1/100 Hemophelia B

Can be transient

HEMOPHILIA

INHIBITOR MANAGEMENT aPCC (FEIBA)

rFVII (NIASTASE)

90 μg/kg q2h x 3 or until bleeding stops

HEMOPHILIA A

CASE

46 yo M, Altered LOC T:38.7 RR:26 BP:76/41 O2: 92% Oozing from IV sites Hematuria from Foley LOOKS SICK

?Investigations

CASE

76

16

40

135

105

6.3

16

131

PTT: 68INR: 2.4

Schistocytes present

LIVER PANEL: Transaminitis and Hyperbilirubinemia

CASE

WORKING DIAGNOSIS?

DIC

DIC

DIC

DIC

SEPSIS ACIDOSIS

TRAUMA HYPOTHERMIA

OBSTETRICAL COMPLICATIONS BURNS

HEPATIC FAILURE AAA

TISSUE DESTRUCTION AMPHETAMINES

MALIGNANCY ENVENOMATIONS

ABO INCOMPATIBILITY HEAT STROKE

DIC

DIC

FDP Elevated Fibrinogen Decreased Factor Levels Decreased D-Dimer Elevated

DISEASE PTT PT/INR PLATELET #

DIC

DIC

MANAGEMENT

Treat cause

Supportive measures

DIC

PLATELET TRANSFUSION

No evidence if not bleeding

Consider if ongoing bleeding

If platelet count < 50 1-2 U / 10 kg / day

FFP/CRYO

No evidence

Usage similar to platelet transfusion considerations

If INR elevated and/or fibrinogen low

DIC

HEPARIN ATIII

DIC

?HEPARIN No controlled trials

Consider use if DIC predominately thrombogenic

May increase bleeding

Start with 500 U/hour, target PTT 45 sec

DIC

?ATIII

Controversial

May have benefit if heparin not also given

DIC

PROTEIN C

DIC

?PROTEIN C

REVIEW

DISEASE PTT PT/INR PLATELET #

vWD N N

HEMOPHILIA N N

DIC

CASE

81 yo M from Africa.

C/O general malaise, fever, cough x 3 days

BIBA from care facility for confusion

Immunization status unknown

O/E

T 38.7 P 130 BP 105/60 RR 20 O2 94% CNS: Confused, agitated. GCS 13 CV: N Resp: N Abdo: Soft. Multiple surgical scars. No

mass.

CASE120

17 124

138

101

4.1

18

140

U/A 6-10 WBC/hpf; 0-2 RBC/hpf.

ECG – Sinus Tach

Howell-Jolly Bodies Present

FEVER AND ASPLENIA

Splenic Function

Active phagocytosis

IgM and Complement production

T lymphocyte reservoir

Scavenger of abnormal cells (eg. spherocytes, inclusion bodies)

FEVER AND ASPLENIA

Asplenia Congenital Surgical removal Autosplenectomy

Increased risk for fulminant bacterial infection Postsplenectomy sepsis (PSS)

FEVER AND ASPLENIA

Organisms are typically encapsulated:

Streptococus pneumoniae

H. influenza

Neisseria meningitidis

FEVER AND ASPLENIA

EVALUATION: Fever must be viewed as PSS

P/E: Toxic and acutely ill

Marked tachycardia and hypotension

Altered LOC

?Primary focus of infection

FEVER AND ASPLENIA

LABS

Left shift +/- bandemia

Thrombocytopenia

DIC

Liver function abnormalities

ABG: Hypoxemia/Hypocarbia

FEVER AND ASPLENIA

MANAGEMENT Regimen:

2 g Ceftriaxone daily 1 g vanco q12h (if multiple drug resistance)

Obtain BC and UC +/- CSF

Low threshold for volume, vasopressor, ventilatory support