Hematology

Question 1

Mucocutaneous bleeding and normal platelet count

Answer 1

Acquired platelet dysfunction (Platelet Function Analyzer-100)

Question 2

Treatment for PE with PESI < 65

Answer 2

Apixaban or rivaroxaban

Question 3

Treatment for inflammatory anemia

Answer 3

None

Question 4

Supplemental treatments in patients with myelodysplastic syndrome requiring frequent transfusions to help decrease transfusion requirements, improve quality of life, and decrease transfusion associated iron overload and alloimmunization

Answer 4

Lenalidomide

Question 5

Multisystem failure and end organ damage commonly involving skin, lungs, GI tract, heart

Answer 5

Hypereosinophilic syndrome

Question 6

Initial lung imaging study to evaluate for pulmonary embolism and pregnant patients

Answer 6

Ventilation-perfusion lung scan

Question 7

Viral infection that preferentially effects erythrocyte precursors in the bone marrow, causing transient pure red cell aplasia in patients with sickle cell anemia

Answer 7

Parvovirus B19

Question 8

Isolated, mild neutropenia found on routine testing in asymptomatic black persons or certain other ethnic groups

Answer 8

Benign ethnic neutropenia

Question 9

Transfusion transmissible pathogen that may be responsible for malaria like symptoms, hemolytic anemia, thrombocytopenia, and abnormal liver chemistry study results

Answer 9

Babesia microti

Question 10

Management to prevent new skeletal related events and improve survival in patients with multiple myeloma requiring therapy

Answer 10

Zoledronic acid

Question 11

Agent to reverse the effects of warfarin anticoagulation in patients experiencing severe bleeding and those requiring urgent surgery

Answer 11

Four-factor prothrombin complex concentrate (4f-PCC)

Question 12

Agent to be given to provide passive immunity against causative organisms in patients with multiple myeloma, hypogammaglobulinemia, and recurrent infections

Answer 12

IVIG

Question 13

Low-grade fever and features of hemolytic anemia after recent transfusion

Answer 13

Delayed hemolytic transfusion reaction (+ direct antiglobulin (Coombs) test)

Question 14

Treatment of choice for patients with bleeding and hypofibrinogenemia secondary to DIC and:or liver disease

Answer 14

Cryoprecipitate

Question 15

Treatment for patients with essential thrombocythemia who are older than 60 years or who have had previous thromboembolic complications

Answer 15

Aspirin + hydroxyurea

Question 16

Gold standard for diagnosing amyloidosis

Answer 16

Biopsy of the affected organ and demonstration of characteristic Apple-green birefringence with Congo red staining

Question 17

Diagnostic test of choice for Waldenström’s macroglobulinemia (Indolent B-cell lymphoma with clonal lymphoplasmacytic infiltration of the bone marrow that secretes IgM in the blood).

Answer 17

Bone marrow biopsy

Question 18

Atypical presentations of rheumatoid arthritis and osteoarthritis, particularly with hook-like osteophytes of the second and third metacarpophalangeal joints

Answer 18

Hemachromatosis

Question 19

Initial therapy for patients with iliofemoral DVT with acute limb ischemia

Answer 19

Thrombolysis

Question 20

Recommended work up for all patients with smoldering multiple myeloma, per the International Myeloma Working group

Answer 20

Whole body MRI to assess for lytic lesions

Question 21

Management for secondary iron overload from chronic transfusions

Answer 21

Oral chelation agents, such as defersirox

Question 22

Initial treatment of chronic myeloid leukemia in the chronic phase

Answer 22

Tyrosine kinase inhibitors, such as imatinib

Question 23

Diagnosis of proximal nocturnal hemoglobinuria

Answer 23

For cytometry, which can detect CD55 and CD59 deficiency on the surface of peripheral erythrocytes or leukocytes

Question 24

Chronic microcytic anemia + normal hemoglobin electrophoresis pattern

Answer 24

Alpha thalassemia trait

Question 25

Management for asymptomatic INR elevation between 4.5 and 10

Answer 25

Withhold warfarin

Question 26

Mucosal or endometrial bleeding, normal PT and aPTT, and normal platelet count with evidence of a qualitative platelet defect

Answer 26

Von Willebrand disease

Question 27

Respiratory distress within six hours of transfusion, positive fluid balance, elevated CVP, elevated BNP, and radiographic findings of pulmonary edema

Answer 27

Transfusion-associated circulatory overload (TACO)

Question 28

Appropriate management for patients with sickle cell disease and acute chest syndrome with respiratory distress requiring mechanical ventilation

Answer 28

Exchange transfusion

Question 29

A monoclonal antibody fragment that binds free and thrombin-bound dabigatran and neutralizes its activity given to patients taking dabigatran and requiring urgent surgery

Answer 29

Idarucizumab

Question 30

Effective treatments for cold agglutinin disease

Answer 30

Cold avoidance and rituximab

Question 31

Management for acquired hemophilia A with inhibitor

Answer 31

Activated factor VII, activated prothrombin complex concentrate, or recombinant porcine factor VIII

Question 32

Most appropriate timing to perform thrombophilia testing

Answer 32

At least two weeks after discontinuation of anticoagulant therapy and not in the acute setting to minimize diagnostic error

Question 33

Diagnosing a myeloproliferative neoplasm in a patient with hepatic vein thrombosis

Answer 33

JAK2 V617F gene mutation

Question 34

Normocytic anemia with a low reticulocyte count and a normal erythrocyte morphology in a patient with underlying CKD

Answer 34

Erythropoietin deficiency

Question 35

RA, splenomegaly, neutropenia

Answer 35

Felty syndrome

Question 36

Treatment for -5q syndrome

Answer 36

Lenalidomide

Question 37

Philadelphia chromosome on the BCR-ABL gene

Answer 37

Chronic myeloid leukemia

Question 38

Management for essential thrombocythemia in high-risk (age > 60, previous thrombosis, platelet > 1.5mil) non-pregnant patients

Answer 38

Hydroxyurea

Question 39

Management for severe acute chest syndrome

Answer 39

Erythrocyte Exchange transfusion

Question 40

Preferred oral anticoagulant in patients with high risk (triple positivity for lupus anticoagulant, anticardiolipin Ab, anti-B2-glycoprotein) antiphospholipid antibody syndrome

Answer 40

LMWH followed by Warfarin

Question 41

Treatment for acute promyelocytic leukemia

Answer 41

All-trans retinoic acid (ATRA)

Question 42

serum monoclonal protein < 3g/dL Bone marrow clonal plasma cells < 10% No end-organ damage

Answer 42

MGUS

Question 43

serum monoclonal protein > 3g/dL Bone marrow clonal plasma cells > 10% No end-organ damage

Answer 43

Smoldering multiple myeloma

Question 44

Management for mild, asymptomatic hereditary spherocytosis

Answer 44

Folate

Question 45

Management for Waldenström macroglobulinemia hyperviscosity syndrome

Answer 45

Plasmapheresis