Hematology Double Jeopardy

Malignant Hematology

100

Which French–American–British classification of ALL typically demonstrates a predominantly uniform population of small blasts with scant cytoplasm, clefting of the nucleus, and variable cytoplasmic vacuolation?

ALL-L1

200

A 63-year-old black man presents with an elevated ESR, 10–30% plasma cells in the bone marrow, M-spike by SPEP, and 4.0 g/dL of serum IgG by radial diffusion. Which plasma cell disorder is implicated?

Multiple Myeloma

300

Burkitt's lymphoma is histologically characterized by which of the following?

"Starry sky" morphology

400

A 14-year-old male presents with a mediastinal mass, a WBC of 110 x 109/L, hepatosplenomegaly, and early central nervous system involvement. Both L1 and L2 morphology are seen, and surface markers CD7, CD2, and CD5 are expressed. Which type of ALL is present?

T-cell

500

This acute leukemia contains primarily abnormal promyelocytes with heavy granulation, and the presence of Auer rods is abundant. The cells react near 100% with Sudan black and peroxidase; however, the reaction is negative for non-specific esterase. Disseminating intravascular coagulation (DIC) is frequently associated with this disorder. What is the FAB classification?

M3

RBC Disorders

100

A patient presents with a hematocrit of 55%, elevated red cell mass, normal arterial oxygen saturation, elevated LAP score, and thrombocytosis. What is the most probable cause of these laboratory findings?

Polycythemia Vera

200

Tissue hypoxia and increased erythropoietic activity is an example of ___________ erythrocytosis. A) Relative B) Stress C) Secondary D) Primary E) None of the above

C) Secondary

300

A strong PAS reaction supports the diagnosis of __________. A) Sickle cell anemia B) CML C) Pernicious anemia D) Erythroleukemia E) None of the above

D) Erythroleukemia

400

Dehydration is an example of __________ erythrocytosis. A) Absolute B) Relative C) Secondary D) Primary E) None of the above

B) Relative

500

5. A patient’s blood smear shows a moderate number of spherocytes and polychromasia. Select the best test to distinguish warm autoimmune hemolytic anemia (WAHA) from hereditary spherocytosis. a. direct antiglobulin test (DAT) b. bilirubin c. haptoglobin d. presence of spherocytes

A) direct antiglobulin test (DAT)

Bleeding and Clotting

100

A 35-year-old woman with fever and severe headaches presents with a markedly decreased platelet count. She has moderate anemia with many red cell fragments on the blood smear as well as polychromasia and nucleated red blood cells. The prothrombin time (PT) and partial thromboplastin time (PTT) are both normal. Which of the following is most likely diagnosis? a. immune thrombocytopenic purpura (ITP) b. thrombotic thrombocytopenic purpura (TTP) c. hemolytic uremic syndrome (HUS) d. disseminated intravascular coagulation (DIC)

B) TTP

200

28. Which of the following is an expected finding in disseminated intravascular coagulation (DIC)? a. elevated D-dimer and fibrin split products b. high platelet count c. low prothrombin time (PT) and partial thromboplastin time (PTT) d. low thrombin time (TT)

A) elevated D-dimer and fibrin split products

300

29. Why does the factor V Leiden mutation increase thrombotic risk? a. makes factor Va resistant to degradation by activated protein C b. makes blood more viscous c. makes factor V more susceptible to activation by thrombin d. decreases the binding of factor Va to the inhibitor antithrombin

A) makes factor Va resistant to degradation by activated protein C

400

6. A patient sample has normal platelet aggregation patterns for all agonists except that there is no reaction with ristocetin. Ristocetin agglutination is not corrected with exogenous von Willebrand factor (VWF). What condition is most likely? a. von Willebrand disease b. storage pool disorder c. Bernard-Soulier syndrome d. Aspirin use

C) Bernard-Soulier syndrome

500

A 28-year-old woman sees her family physician complaining of easy bruising and menorrhagia. She has a platelet count of 60  109/L. All other hematology and routine coagulation tests are normal. She has a platelet-associated IgG autoantibody in her serum. Which of the following is most likely? a. chronic immune thrombocytopenic purpura (ITP) b. thrombotic thrombocytopenic purpura (TTP) c. acute immune thrombocytopenic purpura (ITP) d. hemolytic uremic syndrome (HUS)

A) ITP

Hemolytic Anemia and Thalassemias

100

Warm autoimmune hemolytic anemia accounts for __________ of all cases of autoimmune hemolytic anemia.

70%

200

In a hemolytic anemia, the first normal compensatory bone marrow response will be reflected in which of the following?

Increased reticulocyte count

300

The peripheral blood smear of a patient with warm autoimmune hemolytic anemia will show:

Red cell fragmentation (schistocytes)

400

A 13-year-old boy presents with the following hematologic results: RBC = 5.76 million, Hb, 10.4 g/dL; Hct, 35.9%; MCV (mean corpuscular volume), 63 fL: MCH (mean corpuscular hemoglobin), 18.2 pg; MCHC (mean corpuscular hemoglobin concentration), 29%; and RDW (red blood cell distribution width), 13.5. Morphology includes mild anisocytosis and occasional target cells. All iron studies are normal. Electrophoresis on cellulose acetate at pH 8.4 indicates an increase in HbF and HbA2. What thalassemia syndrome is evident?

Beta Thalassemia

500

9. Secondary cold-reactive autoimmune hemolytic anemia is found most often in patients with: a. hemolytic disease of the newborn caused by anti-A or anti-D b. acute and chronic myeloid leukemias c. Mycoplasma pneumoniae or infectious mononucleosis d. Hereditary spherocytosis or hereditary elliptocytosis

C) Mycoplasma penumoniae or infectious mononucleosis

Miscellaneous

100

Which cytochemical stains show strong positivity in acute monocytic leukemia (M5)?

Non-specific Esterase

200

A 5-year-old girl presents with anemia, neutropenia, thrombocytopenia, lymphoblasts, and prolymphocytes. Cytochemical examination shows myeloperoxidase and SBB negative, and a positive for TdT. This hematologic picture is consistent with:

ALL

300

Which type of MDS demonstrates a mild decreased white blood cell (WBC) count (3.9 x 10/L), increased erythropoiesis and normal numbers of blast cells in the marrow, and <15% sideroblasts?

Refractory Anemia (RA)

400

A 35-year-old man presents with anemia, neutropenia, thrombocytopenia, myeloblasts with the presence of Auer rods, and one or two distinct nucleoli and promyelocytes. Cytochemistry examination demonstrates peroxidase and Sudan black B (SBB) positive and TdT terminal deoxynucleotidyl transferase (TdT) negative. This hematologic picture is consistent with:

AML

500

A patient is admitted to the ER with the following laboratory results: WBC count 200 x 109/L, blast count 13%, Hgb = 9 g/dL, decreased/absent LAP score. Which condition should the physician suspect?

CML