1.
A 30-year-old woman has H/O mild fatigue for many years. O/E Palpable spleen tip. Labs- Hb 11.1 g/dL, Hct 28.8%, MCV 83fL, platelets 229,000/µL, WBC count 7340/µL. Corrected retic. Count-12%. PBS- Small RBCs that lack central pallor. Osmotic fragility of RBCs is increased. Coombs test is negative. Defects in which of the following are involved in the pathogenesis of this disorder
A. 
B. 
C. 
D. 
E. 
Iron utilization from iron stores
2.
The patients described in the previous question is at an increased risk of developing which of the following conditions?
A. 
B. 
C. 
D. 
E. 
3.
A 10-yr-old black girl is brought to the ER with severe pain in her chest, abdomen and bones. O/E- Jaundice and anemia. CBC shows abnormal shaped RBCs and Howell-Jolly bodies. Labs - Increased amount of an abnormal Hb in her peripheral blood. The child’s chest and bone pain symptoms are caused by which of the following mechanisms?
A. 
B. 
C. 
D. 
4.
In the patient described in the previous question, which of the following is the most likely mechanism for initiation of her pulmonary problems?
A. 
B. 
Increased RBC adhesion to endothelium
C. 
D. 
Increased osmotic fragility of RBCs
E. 
Reduced expression of adhesion molecules on endothelial cells
5.
A 10-month-old boy presents with failure to thrive. O/E- Jaundice. CBC- Microcytic hypochromic anemia. Most of the Hb is HbF type. PBS is shown. BM aspirate shows marked erythroid hyperplasia and increased iron stores. What is your diagnosis?
A. 
B. 
C. 
D. 
6.
What is the pathogenesis of anemia seen in the patient just described?
A. 
B. 
Abnormal spectrin assembly
C. 
D. 
Decreased beta-globin chain synthesis
E. 
Impaired utilization of iron from storage sites
7.
A 16-year-old boy has had a low energy level for as long as he can remember. O/E- Palpable spleen tip. CBC shows Hb- 8.8 g/dL, Hct 24.1%, MCV 65 fL, platelet count 187,000/µL, WBC count 7400/µL. His serum ferritin is 3740 ng/mL (Normal-10-200µg/L). BM biopsy reveals a myeloid : erythroid ratio of 1:4 with 4+ stainable iron. Which of the following is the most likely diagnosis?
A. 
B. 
C. 
D. 
E. 
8.
A 19 year old primigravida of Southeast Asian ancestry gives birth at 35 wks gestation a male infant. O/E infant is markedly hydropic. Lab. studies show his hematocrit is 17% and PBS reveals numerous nucleated red blood cells. RBCs show marked anisocytosis and poikilocytosis. Which of the following diseases is most likely to be present in this infant?
A. 
B. 
C. 
D. 
Hereditary elliptocytosis
9.
A 49-year-old female presents with signs of anemia and states that every morning her urine is dark. Work-up reveals that her RBCs lyse in vitro with acid (positive Ham’s test). What is the best diagnosis for this patient?
A. 
Warm, autoimmune hemolytic anemia
B. 
Paroxysmal nocturnal hemoglobinuria
C. 
Cold-agglutinin autoimmune hemolytic anemia
D. 
10.
A 25-year-old African-American man is given anti-malarial prophylaxis for a trip to West Africa. Over the next week he develops increasing fatigue. O/E - No abnormal findings. Labs- Hematocrit -30%. PBS shows RBCs with numerous Heinz bodies. There is a family history of this disorder, with males, but not females, affected. Which of the following is the most likely diagnosis?
A. 
B. 
C. 
D. 
E. 
11.
Which of the following underlying conditions would you most likely expect to be present in a 27-year old female with the peripheral blood smear shown here:
A. 
B. 
Anemia of chronic disease
C. 
D. 
E. 
12.
A 38-year-old woman has become increasingly fatigued for the past 3 months.O/E- Purpuric areas of skin on her trunk and extremities. No hepato-splenomegaly/LN. Labs- Hb 6.8 g/dL, Hct 20.7%, MCV 91 fL, platelet count 28,760/µL, and WBC count 1940/µL. Which of the following is the most likely diagnosis?
A. 
B. 
Chronic myelogenous leukemia
C. 
D. 
E. 
13.
A 32-yr-old man presents with fever, recurrent URIs and chronic fatigue. He is an immigrant from Russia and has worked in a benzene factory. O/E- Petechiae seen, no LN/splenomegaly. CBC shows pancytopenia. Retic. count is low. BM biopsy shows a hypocellular marrow with increased fat. Which of the following is the most likely underlying mechanism of the patient’s condition?
A. 
B. 
Impaired globin chain synthesis
C. 
D. 
14.
A 38-year-old woman has a history of rheumatoid arthritis. She is currently receiving immunosuppressive therapy including corticosteroids. O/E - Minimal erythema, warmth, and swelling of the small joints of her hands. Lab. studies show Hb 10.1 g/dL, Hct 30.4%, MCV 74 fL, WBC count 6500/µL, and platelet count 137,000/µL. Retic. Count-1.5%. She has a serum iron of 28 µg/dl and TIBC of 200 µg/dl . Her serum ferritin is 420 ng/mL. Which of the following is the most likely diagnosis?
A. 
B. 
C. 
Anemia of chronic disease
D. 
Auto-immune hemolytic anemia
15.
A 38-yr-old man presents with fatigue. He has a 3-yr history of TB. CBC shows a microcytic hypochromic anemia. Blood profile shows low serum iron, low iron binding capacity and increased serum ferritin. Which of the following explains the pathogenesis of anemia in this patient?
A. 
B. 
Impaired utilization of iron from storage sites
C. 
Synthesis of structurally abnormal globin chains
D. 
E.