June 20th, 2010TJ O’Neill

Hem/Onc Board Review

Question 23What happened longest ago?

A. Barry Bonds broke Hank Aaron’s HR RecordB. The last Harry Potter book was releasedC. TJ last took care of a Med E patient in any

capacityD. D/c summaries transitioned from paper to

electronic

Question 23What happened 1065 days ago? ANSWER C

A. Barry Bonds breaks Hank Aaron’s RecordAugust 7th, 2007

B. The last Harry Potter book was releasedJuly 25th, 2007

C. TJ last took care of a Med E patient in any capacityJuly 23rd, 2007

D. D/c summaries transitioned from paper to electronicAugust 2007

Question 1A 72 yo man is hospitalized because of dyspnea,

anginal chest pain and new-onset anemia. CLL was diagnosed 2 years ago. He has not been treated or transfused.

On exam the pt is pale,bp 110/80, hr 112, rr 24. Diffuse cervical, axillary, and inguinal LAD s present. There is no JVD, lungs are clear, abdominal exam shows splenomegaly, no edema.

Hgb 6.0, WBC 55,000 w/ 90% lymphs, Plt 115,000, Retic count 12%. Tbili 2.8, Dbili 0.6.

Direct coombs Postive for IgG, T/S is A positiveSmear shows microspherocytosis. Steroids are started and 2 type and crossed units

are ordered but no compatible units are available

Question 1Which is the most appropriate management at

that time

A. Begin EPOB. Schedule splenectomyC. Transfuse one unit of A-positive erythrocytesD. Transfuse one unit of O-positive

erythrocytesE. Withhold transfusion until compatible unit of

blood is available

Answer C- Transfuse A negative blood

Symptomatic anemia 2/2 CLL- hemolytic confirmed by Coombs test and microspherocytes

Given lack of pregnancy or prior xfusions very unlikely to have alloimmunization to erythrocyte antigens. Because of broad specificity of autoantibodies in patients with autoimmune hemolytic anemia most donor cells with be technically incompatible on cross-match

EPO not indicated given appropriate retic countRarely will splenectomy be needed

Steroids would be given as well but take time to work

Question 742 y/o man is hospitalized because of hematuria.

The pt has a mechanical mitral valve and has been taking warfarin 5mg/day

On exam he is alert and pale. BP 105/65, HR 96, rr 16, lungs are clear.

Labs: Hemoglobin 8.0, Plt 200,000, INR 7.0, aPTT 28, urinalysis shows gross blood

3 units of FFP are transfused over the next 4 hours. Halfway through pt develops severe dyspnea w/o chest pain or cough. No fever, BP 115/70, HR 104, RR 28, sat 86%.

Pt has bibasilar crackles, no s3 or edema. BNP normal, cxr shows bilateral infiltrates

Question 7Which is the most likely diagnosisA. AnaphylaxisB. Aspiration pneumoniaC. Pulmonary EmbolismD. Heart Failure E. Transfusion related acute lung injury

Answer E- TRALIOccurs generally within 6 hours of plasma rich

blood products. Due to donor antileukocyte Ab reacting with

recipient leukocytes causing leukocyte plugs to form in pulmonary capillary bed

Clinically improve in 2-3 daysManagement is stop transfusion and give

supportive care

Transfusion associated circulatory overload (TACO) unlikely given exam and normal BNP

Other transfusion syndromesAcute hemolytic transfusion reaction- within 24 hours.

Usually due to ABO incompatibilityDelayed transfusion reaction within 7-14 days, more

common than acute. Characterized by fever, jaundice, unexplained drop in Hgb, labs show hemolysis. Generally due to a remote transfusion event or pregnancy and low Ab levels

Transfusion related sepsis- PlateletsFebrile non-hemolytic- due to donor cytokines, consider

leukoreducedAllergic reactions- Generally mild unless recipient IgA

negativeTransfusion associated GVHD- chemo, blood from 1st

degree relatives, premature infants, need irradiated blood. Can be fatal

Question 10Is this kid awesome?

A. YesB. NoC. Is that a young Kubal?

Answer: Yes

Question 858 y/o man is evaluated with increasing fatigue

over 2 months. Pt has HTN, HLD treated with lisinopril, atorvastatin. A sister has hypothyroid

On exam, Temp normal, BP 135/80, HR 72, RR 18. No LAD or edema. The spleen is palpable.

Hgb 12.1, WBC 55,200, Plt 105,000. Smear shows increased granulocytic cells in all phases but no Auer rods. BM exam shows hypercellular marrow (80% cellularity) with marked granulocytic hyperplasia, left shift in the granulocytes and 3% myelocytes. Cytogenetic testing shows BCR/ABL translocation

Question 8Which is most appropriate next stepA. Administer ImatinibB. HLA typing of pt and his sisterC. LeukopheresisD. Observation and monthly f/u

Answer AThe pt requires Imatinib (Gleevec). CML results from balanced translocation of

chromosomes 9:22 creating BRC:ABL. Chronic CML pts have less than 10% blasts in marrow and blood. However eventually as disease progresses blast count may increase and progress to blast crisis

HSCT best reserved for very young or refractoryLeukopheresis for blast count > 50,000Would not observe because best chance to treat

is during chronic phaseDasatinib and nilotinib are new agents

Question 1166 y/o woman evaluated for 1 month of gradually

increasing HA, blurred vision, and episodes of confusion. PMHx and family hx are unremarkable. No meds

She is afebrile, BP 150/80, HR 90. Retinal exam shows dilated, segmented, tortous retinal veins. She has an S3 and clear lungs. Abdominal exam shows hepatosplenomegaly.

Hgb 8.5, WBC 14,000 with 80% lymphs, IgM 4800 mg/dL, LD 80

CXR shows cardiac enlargement. CT of chest and abdomen shows enlarged liver, spleen and many enlarged lymph nodes. BM biopsy reveals 70% replacement with lymphoplasmocytic cells

Question 11Which is the next best therapeutic optionA. Blood transfusionB. FurosemideC. Plasma exchangeD. IVIG

Answer CPt has lymphoplasmacytic lymphoma

(Waldenstrom macroglobulinemia). Sx include blurred vision, fatigue, mucosal

bleeding, headache, heart failure, and altered mentation. Plasma exchange is indicated acutely followed by Ritux or fludaribine.

Fundoscopic exam may show engorged retinal veins

Blood transfusion is contraindicated

Waldenstrom Macroglobulinemia

Proliferation of B lymphocytes that show maturation to plasma cells

Lymphoplasmacytic infiltration of BM

Elevated Igm, LAD, anemia, neuropathy, organomegaly, IgM monoclonal gammopathy and hyperviscocity syndrome

Question 1226 y/o woman admitted for DVT. No PmHx and

only takes OCP. Mild scleral icterus, 37.2, BP 110/67, HR 100, RR

16, abdominal exam shows mild splenomegalyHgb 10, WBC 2700, Plt 42,000, Retic 8%, MCV 70,

Tbili 5.0, Dbili 0.8, LDH 1126AST, ALT, AP are normal. BM bx shows

hypocellular marow, absent iron stores and signs of early myelodysplasia. Direct and indirect cooms are negative. What next?

A. Flow cytometryB. Hgb electrophoresisC. Osmotic fragility studyD. Parvovirus B19 serology

Answer APancytopenia, hemolytic anemia, and thrombosis,

concerning for PNH.PNH caused by loss of GPI linked proteins on cell

surface causing increased RBC sensitivity to complement

CD55 and CD59 deficiency can be detected by flowIron deficiency is common due to chronic iron loss

in urineThrombosis may occur 2/2 loss of anticoagulants

linked to GPI anchorsEculizamab, Ab to C5, is a recently developed

therapy

Question 29Which evil organ system caused this problem?

A. KidneysB. LungsC. BloodD. Rheumatological

Answer C, blood

Question 1578 y/o woman has 3 month hx of increasing fatigue. No

med hx, no meds. Afebrile, HR 72, BP 130/80, RR 16. Pt appears pale.

Hgb 7.8, WBC 2,800 ANC 1,200, Plt 560K, Epo 600BM bx shows hypercellular marrow with erythroid

hyperplasia and dysplasia of the erythroid and granulocyte series. Megakaryocytes are increased with many hypolobulated cells. Iron stores are normal. Cytogenics studies show deletion of long arm of chromosome 5 (5q-)

Which is the most appropriate A. Azacitidine B. Danazol C. LenalidomideD. G CSF and EPO

Answer CDysplasia of erythocyte, granulocyte, or megakaryocyte

lineages in the setting of hypocellular bone marrow suggest MDS.

Detection of clonal abnormalities in xsomes 3,5,7,8,17 support the diagnosis.

5q- is characterized by elevated Plts and anemia, typically elderly women with a indolent course. Associated with xfusion dependant anemia, low incidence of neutropenia or thrombocytopenia.

This subtype responds well to leulinomide, thalidomide analogue

Azacitidine is better for other types of MDSDanazol can treat MDS associated anemia but has many side

effectsEPO level too high to give EPO

Question 1854 y/o man evaluated for increased lethargy and

vague abdominal symptoms x2 weeks. No PMHx, FHx. No smoking, no meds

BP 136/82, P 90, RR 18, Sat 99% RA. He has no clubbing or cynanosis. Abdomen is soft, no hepatosplenomegaly.

Hgb 20.2, Plt 312,000, WBC 8200, EPO 35Which is most appropriate next diagnostic test?A. Abdominal ultrasoundB. EchocardiogramC. JAK2 mutation analysisD. Erythrocyte mass study

Answer APt with elevated EPO levels in the setting of

erythrocytosis usually either exogenous EPO or chronic hypoxia

Given normal sat, RCC is the most likely dx. Only 1-5% of pt with RCC have erythrocytosis, 20-80% have anemia.

P vera would have a low EPO level

Causes of Secondary Polycythemia and Elevated EPO

LevelsTumor related increased serum EPO levels

RCC, hepatocellular carcinoma, uterine fibroids

HypoxemiaCOPD, OSA, altitude

Increased carboxyhemoglobinSmoking

Abnormal hemoglobinHigh oxygen-affinity hgb

Question 2419 y/o AAM evaluated 2 days of arm, leg and back

pain. PMhx positive for similar episodes of pain but not this severe and anemia of unclear cause. FHx positive for anemia. ROS + poor exercise tolerance, no meds, no allergies.

Exam +scleral icterus, 99.0, 126/62, 112, 18, BMI 22. No splenomegaly or hepatomegaly

Hgb 10.2, WBC 8600, MCV 70, Tbili 5.7, Dbili 0.3. Smear shows rare sickles erythrocytes and target cells

Hgb Electrophoresis. Hgb S 67%, Hgb F 3%, A 25%, A2 5%

Diagnosis? A. Sickle cell trait B. S+ thalessemia C. Hgb SC disease, D Hgb S w/ hereditary persistence of fetal hemoglobin

Answer BS+ thalessemia is characterized by

hemoglobin S> 60%, elevated A2 and microcytosis. Generally milder than SS disease

Sickle cell trait, AS, have S level <50% and higher A level as well as normal sized RBCs

SC disease would be 50:50 Hgb S to CSS disease have <70% S and >30% Hgb F

Question 3627 y/o man evaluated for new onset anemia

found during routine CBC. Pt is s/p kidney xplant for hereditary nephritis (Alport’s) 2 months ago. Meds are cyclosporine, mycophenolate, prednisone, Bactrim. No other medical hx

On exam pale conjunctiva, 37.5, 136/82, 112, 18. Petichiae on lower extremities. R pelvic kidney is nontender with healed scar

Hgb 8.1, Plt 120,000, WBC 8200, LDH 1430, Cr 3.2, direct and indirect Coombs are negative

A smear is obtained.

Which medication is most likely responsibleA CyclosporineB MycophenolateC PrednisoneD. Trimethoprim- sulfamethoxazole

Answer APt has cyclosporine induced HUSMAHA with negative Coombs. Would stop cyclosporine, PLEX is not well

studied for this syndrome

Mycophenolate and Bactrim would cause myelosuppression but not MAHA

Question 4757 y/o woman evaluated for 1 week of swelling and

pain in LLE. 2 normal pregnancies, no miscarriages. No PMhx or hx of clots

DVT is confirmed on u/s and pt is started on unfractionated heparin infusion. Warfarin 5mg/day is also started

Which is the most appropriate heparin therapy?A. Minimum 5 days, 2 INR measurements >2, 24 hr

apartB. Minimum 2 weeks, with 4 INR measurements >2,

24hr apartC. Min 3 days with 1 INR measurement >2D Min 24 hours with 1 INR measurement of >2

Answer ANeed >24 of fully therapeutic Warfarin to allow

further reduction of prothrombin, the Vit K dependant clotting factor with the longest half-life (60hr)

Question 5050 y/o woman with advanced multiple myeloma

diagnosed 6 months ago comes for follow up. Treatment includes daily thalidomide and pulse dexamethasone. She feels well.

Labs show serum monoclonal protein concentration of 3.0 g/dL. Hgb concentration, serum calcium, and renal function are normal. BM aspirated shows reduction in plasma cells from 50% to 10%

Which of the following is most appropriate trx to optimize this patients disease free and overall survival.

A. Autologous stem cell transplantationB. Continuation of oral thalidomideC. Initiate parenteral bisphosphonatesD. Initiate oral melphan

Answer AThalidomide plus Dex is first line for pts less

than 65 who are candidates for xplant. Response to chemo is 65-75% which should be followed by stem cell transplant.

Oral melphan is used for MM but can impair collection of stem cells