Hema

Question 0

Diagnostic test for PNH

Answer 0

Flow cytometry

Question 1

Markers absent on erythrocytes in patients with PNH

Answer 1

CD55 and CD59

Question 2

Virus that causes chronic pure red cell aplasia in patients with AIDS and transient transient aplastic crisis in patients with sickle cell disease

Answer 2

Parvovirus B19

Question 3

Treatment for PNH that reduces intravascular hemolysis, hemoglobinuria, and the need for transfusion

Answer 3

Eculizumab

Question 4

First-line therapy in young patients with aplastic anemia (less than 40 years)

Answer 4

HSCT

Question 5

Treatment for patients with AIDS and chronic parvovirus B19 infection

Answer 5

IV immune globulin

Question 6

Most frequent cause of normocytic anemia

Answer 6

Inflammatory anemia

Question 7

Serum ferritin level that rules out iron deficiency, even with inflammation

Answer 7

More than 100 ng/mL

Question 8

Percentage of patients with iron deficiency with normal MCV

Answer 8

33%

Question 9

Most common cause of microcytic anemia

Answer 9

Iron deficiency

Question 10

First morphologic sign of iron deficiency

Answer 10

Hypochromia (decreased MCHC)

Question 11

Most useful test in the diagnosis of iron deficiency

Answer 11

Serum ferritin

Question 12

Standard treatment (and duration) for iron deficiency

Answer 12

Oral iron for 6 months

Question 13

Homocysteine and methylmalonic acid levels in vitamin B12 deficiency

Answer 13

Both increased

Question 14

Homocysteine and methylmalonic acid levels in folate deficiency

Answer 14

Elevated homocysteine, normal methylmalonic acid

Question 15

Initial therapy for warm-antibody and cold agglutinin autoimmune hemolytic anemia

Answer 15

Corticosteroids

Question 16

First-line therapy for hereditary spherocytosis and transfusion-dependent thalassemias

Answer 16

Splenectomy

Question 17

Standard treatment for severe thalassemia and severe PNH

Answer 17

Stem cell transplantation

Question 18

Supplement needed by all patients with hemolytic anemia

Answer 18

Folic acid

Question 19

Best time to test for enzyme concentrations in G6PD deficiency

Answer 19

2-3 months after a hemolytic event

Question 20

Diagnostic test for sickle cell syndromes

Answer 20

Hemoglobin electrophoresis

Question 21

Transfusion target in sickle cell disease to avoid hyperviscosity

Answer 21

Hemoglobin less than 10 g/dL

Question 22

Hemoglobin seen in alpha-thalassemia with three-gene deletion

Answer 22

Hemoglobin H (4 beta)

Question 23

Hemoglobin seen in alpha-thalassemia with four-gene deletion resulting in hydrops fetalis

Answer 23

Hemoglobin Bart’s

Question 24

Also known as Cooley anemia

Answer 24

Beta-thalassemia major

Question 25

Drug-induced ITP is most often linked to these drugs

Answer 25

Quinine or quinidine

Question 26

First-line therapy for ITP

Answer 26

Corticosteroids

Question 27

Thrombopoiesis-stimulating agents used in refractory cases of ITP

Answer 27

Eltrombopag, romiplostim

Question 28

Most common cause of thrombocytopenia in a pregnant patient

Answer 28

Gestational thrombocytopenia

Question 29

Treatment for TTP

Answer 29

Plasma exchange

Question 30

Gold standard for diagnosis of HIT

Answer 30

Serotonin release assay

Question 31

Hallmark of primary hemostasis failure

Answer 31

Mucocutaneous bleeding (epistaxis, gingival bleeding, easy bruising, menorrhagia)

Question 32

Treatment for intracranial bleeding due to excessive warfarin administration

Answer 32

Recombinant human factor VIIa or prothrombin complex concentrate

Question 33

Most common inherited bleeding disorder

Answer 33

von Willebrand disease

Question 34

Treatment for vWD with mild to moderate bleeding or before minor invasive procedures

Answer 34

DDAVP

Question 35

Transfusion reaction diagnosed by an assay for antibodies to human platelet antigen-1a

Answer 35

Posttransfusion purpura

Question 36

Treatment for posttransfusion purpura

Answer 36

IV immune globulin

Question 37

Prevention for transfusion-associated GVHD

Answer 37

Gamma irradiation of cellular blood components

Question 38

Most common defects leading to thrombophilia (2)

Answer 38

Factor V Leiden and prothrombin gene mutations

Question 39

Most common hereditary thrombophilia in white populations

Answer 39

Factor V Leiden mutation

Question 40

Most common acquired thrombophilia

Answer 40

Antiphospholipid syndrome

Question 41

Most common malignant plasma cell dyscrasia

Answer 41

Multiple myeloma

Question 42

Presence of hepatomegaly in multiple myeloma should prompt evaluation for this condition

Answer 42

Amyloidosis

Question 43

Preferred pretransplant induction chemotherapeutic regimen for multiple myeloma

Answer 43

Dexamethasone plus lenalidomide (or thalidomide)

Question 44

Treatment for multiple myeloma in patients who are not transplant candidates

Answer 44

Melphalan and prednisone

Question 45

Vaccine indicated for multiple myeloma patients treated with bortezomib

Answer 45

Varicella-zoster vaccine

Question 46

Drug to be avoided in multiple myeloma patients who are candidates for stem cell transplantation

Answer 46

Melphalan

Question 47

Most common myeloproliferative disorder

Answer 47

Essential thrombocythemia

Question 48

Treatment for erythromelalgia in patients with essential thrombocythemia

Answer 48

Low-dose aspirin

Question 49

Patients with essential thrombocythemia who do not require therapy

Answer 49

Age less than 60, asymptomatic, and platelet less than 1.5 million

Question 50

Tests to distinguish polycythemia vera from secondary erythrocytosis (2)

Answer 50

JAK2 mutation, low erythropoietin

Question 51

Hematocrit levels to diagnose polycythemia vera

Answer 51

Men >60%, women >56%

Question 52

Treatment of choice for patients with polycythemia vera and at high risk for thrombosis (2)

Answer 52

Hydroxyurea and phlebotomy

Question 53

Goal hematocrit in therapeutic phlebotomy for polycythemia vera

Answer 53

Less than 42% in women, less than 45% in men

Question 54

Intervention contraindicated in myelofibrosis with myeloid metaplasia because it does not improve mortality and increases risk of progression to leukemia

Answer 54

Splenectomy

Question 55

Treatment for myelofibrosis with myeloid metaplasia in patients less than 60 years

Answer 55

Allogeneic stem cell transplantation

Question 56

Specific treatment for 5q-syndrome subtype of MDS

Answer 56

Lenalidomide

Question 57

Most common leukemia encountered in adults

Answer 57

Chronic lymphocytic leukemia

Question 58

An aggressive relapse of CLL with B symptoms, massive lymphadenopathy, and hepatosplenomegaly

Answer 58

Richter transformation

Question 59

Single drug treatment for stage III or IV CLL in older patients

Answer 59

Chlorambucil

Question 60

Markers detected on flow cytometry in hairy cell leukemia

Answer 60

CD11 and CD103

Question 61

Treatment for hairy cell leukemia

Answer 61

Cladribine (purine analog)

Question 62

Of all the leukemias, this will most likely involve significant thrombocytopenia with bleeding, bruising, petechiae, and infection

Answer 62

AML

Question 63

Physical examination findings that suggest an alternative or concomitant diagnosis other than AML

Answer 63

Lymphadenopathy and hepatosplenomegaly

Question 64

Blood smear findings pathognomonic of AML

Answer 64

Auer rods

Question 65

Three cytogenetic abnormalities that carry a good prognosis

Answer 65

t(8;21), t(15;17), inv(16)

Question 66

Percentage of bone marrow blasts to confirm a diagnosis of AML

Answer 66

More than 20%

Question 67

Treatment for acute promyelocytic leukemia

Answer 67

ATRA

Question 68

Drug combination that can induce higher remission rates in AML

Answer 68

Cytatabine and an anthracycline (daunorubicin or idarubicin)

Question 69

Drug used to induce remission in relapsed older adult patients with CD33+ AML

Answer 69

Gemtuzumab ozogamicin

Question 70

Treatment for retinoic acid syndrome

Answer 70

Dexamethasone