IM Hematology

Question 1

What is the physiologic cause of hemolytic anemia in G6PD deficiency?

Answer 1

Reduction in glutathione levels in red cells

Question 2

What is the inheritance pattern of G6PD deficiency?

Answer 2

X-linked recessive

Question 3

What ethnicities is G6PD deficiency most commonly seen in?

Answer 3

African and Mediterranean

Question 4

Name some drugs that are likely to lead to acute hemolysis in patients with G6PD deficiency?

Answer 4

Oxidative drugs such as primaquine, aspirin, dapsone, quinidine, sulfonamides, and nitrofurantoin

Question 5

What is seen on peripheral blood smear that is consistent with a hemolytic episode of G6PD deficiency?

Answer 5

Heinz bodies and bite cells

Question 6

Is thalassemia a macrocytic or microcytic anemia?

Answer 6

Microcytic

Question 7

What ethnicity is alpha thalassemia commonly found in?

Answer 7

Southeast Asian and Chinese

Question 8

What ethnicity is beta thalassemia most commonly found in?

Answer 8

African and Mediterranean

Question 9

How many types of alpha thalassemia are there? What are they?

Answer 9

4 types:
Carrier - 1  alpha gene deletion
Minor - two alpha gene deletions
Hb H disease - 3 alpha gene deletions
Hydrops fetalis - 4 alpha gene deletions

Question 10

What chromosome is the alpha gene found on?

Answer 10

Chromosome 16

Question 11

What is the inheritance pattern of sickle cell anemia?

Answer 11

Autosomal recessive

Question 12

At what age does sickle cell anemia begin to present? Why?

Answer 12

Around 6 months, when fetal hemoglobin is replaced with HbS.

Question 13

What is the most common cause of death due to sickle cell anemia?

Answer 13

Acute chest syndrome

Question 14

What is the definitive diagnostic study for sickle cell anemia?

Answer 14

Electrophoresis

Question 15

What is the only pharmacologic agent that has consistently demonstrated improvements in episodes of pain and survival rates for patients with sickle cell anemia?

Answer 15

Hydroxyurea

Question 16

What is the mechanism of action of hydroxyurea?

Answer 16

Increases production of hemoglobin F and reduces production of hemoglobin S

Question 17

Why are patients with sickle cell anemia prone to infection by encapsulated organisms?

Answer 17

They have a dysfunctional spleen

Question 18

What anemias a megaloblastic anemias?

Answer 18

Vitamin B12 deficiency and folate deficiency anemias

Question 19

What is the most common cause of vitamin B12 deficiency anemia?

Answer 19

Strict veganism

Question 20

What is the most common cause of vitamin B12 deficiency?

Answer 20

Pernicious anemia

Question 21

What is pernicious anemia?

Answer 21

Autoimmune destruction of parietal cells resulting in decrease in production of intrinsic factor causing B12 deficiency

Question 22

What is the pathophysiologic cause of neurologic symptoms in vitamin B12 deficiency anemia?

Answer 22

Accumulation of methylmalonic acid causes degeneration of the spinal cord

Question 23

What type of anemia classically presents with koilonychia and pica?

Answer 23

IDA

Question 24

Deficient activity of the plasma enzyme ADAMTS13 causes what hematologic disorder?

Answer 24

Thrombotic thrombocytopenic purpura

Question 25

What is the classic pentad of symptoms associated with thrombotic thrombocytopenic purpura?

Answer 25

Fever, anemia, thrombocytopenia, kidney insufficiency, and neurological disturbances

Question 26

Which microangiopathy is Shiga toxin-mediated and results after ingestion of undercooked ground beef contaminated with Escherichia coli?

Answer 26

Hemolytic uremic syndrome.

Question 27

What is the most common pathologic fracture associated with multiple myeloma?

Answer 27

Vertebral fractures

Question 28

A monoclonal protein spike on serum protein electrophoresis is commonly seen in _____?

Answer 28

Multiple myeloma

Question 29

What protein is commonly seen in urine in multiple myeloma?

Answer 29

Bence Jones protein

Question 30

What are the expected findings in radiographs of the skull in multiple myeloma?

Answer 30

Punched out lesions

Question 31

What facial bone is associated with necrosis resulting from long-term bisphosphonate use?

Answer 31

Jaw.

Question 32

What will a peripheral blood smear show in a patient with multiple myeloma?

Answer 32

Rouleaux formations

Question 33

Hemophilia A results from deficiency of what clotting factor?

Answer 33

Factor VIII

Question 34

Hemophilia B results from lack of what clotting factor?

Answer 34

Factor IX

Question 35

What medication can be given to patients with hemophilia A to increase serum level of factor VIII?

Answer 35

Desmopressin acetate

Question 36

What factor enhances platelet aggregation and adhesion?

Answer 36

Von Willebrand factor.

Question 37

What is the inheritance pattern of hemophilias?

Answer 37

X-linked recessive