Heme Questions

What is the most accurate test to diagnose Aplastic anemia?

Bone marrow aspiration

Which of the following is the most common inherited cause of hypercoagulability in the US?

Factor V Leiden mutation

Folate deficiency is classically associated with which type of anemia?

Macrocytic

Which of the following is most helpful in the management of Sickle cell crises?

Analgesics

Which of the following has been shown to be most effective in preventing the recurrence of strokes in children with Sickle cell disease?

Chronic transfusion therapy

What is the first line of management in a 7-year-old boy with decreased urine output and tea-colored urine?

IV fluids, electrolyte replacement and admission for supportive therapy

Immune thrombocytopenic purpura (ITP) is what type of hypersensitivity reaction?

Type II (cytotoxic)

Autosplenectomy is a common feature of which of the following?

Sickle cell disease

Von Willebrand factor binds to which of the following?

Glycoprotein GP1b receptor on platelets

Plummer-Vinson syndrome is characterized by?

Iron deficiency anemia + dysphagia + cervical esophageal web

During genetic counseling of a couple who both have the sickle cell trait, what are the chances of having a child with sickle cell disease?

25%

Which disorder is associated with decreased production of Hepcidin?

Hereditary hemochromatosis

Which of the following is an antiplatelet agent?

Clopidogrel

Which is abnormal in patients with immune thrombocytopenic purpura?

Platelet count

What is the most likely diagnosis for a 27-year-old female with jaundice and Coombs positivity?

Warm autoimmune hemolytic anemia

Increased hemosiderin deposition in the liver is hallmark of which condition?

Hereditary hemochromatosis

Which is not a classic lab finding in patients with Polycythemia vera?

Increased serum erythropoietin levels

What describes the main pathophysiologic mechanism for thrombotic thrombocytopenia purpura?

Deficiency in ADAMTS13, a von Willebrand factor cleaving protease

What is the most likely diagnosis for a patient with decreased hemoglobin, hematocrit, low MCV, and low serum ferritin?

Iron deficiency anemia

Which lab value is increased in a patient with iron deficiency anemia?

RBC distribution width

What is the underlying etiology for the abdominal pain in a 15-year-old male with target cells and Howell-Jolly bodies?

Sickle cell disease

Which neurological complication is not associated with pediatric patients with sickle cell disease?

Optic neuritis

What is the initial management of choice for a 19-year-old male with autoimmune thrombocytopenic purpura?

Glucocorticoids

Which medication is not associated with causing aplastic anemia?

Diphenhydramine

What is most likely in the medical history of a 13-year-old boy with a painful erection?

Sickle cell disease

Which is not a classic finding of hemolytic anemia?

Decreased reticulocytes

What is the next recommended step for a 71-year-old male with bone pain and anemia?

Plain radiographs of the spine

What is the likely diagnosis for a 60-year-old female with decreased exercise tolerance, increased MCV, and normal methylmalonic acid?

Folate deficiency

In Waldenstrom macroglobulinemia, serum protein electrophoresis shows a spike of which immunoglobulin?

IgM

What is the most appropriate next step for a 5-year-old boy with decreased activity and normocytic normochromic anemia?

Bone marrow biopsy

What is the hallmark of lead poisoning in RBCs?

Basophilic stippling

What is the first-line management of Polycythemia vera without evidence of thrombosis?

Phlebotomy

What is the most likely etiology for chronic abdominal pain in a child after exposure to renovation dust?

Lead poisoning

Which group is least at risk for iron deficiency anemia?

30-year-old male with history of asthma

What is the classic type of anemia in sickle cell disease?

Normocytic normochromic