IM Hematology Flashcards

1
Q

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

A

Reduction in glutathione levels in red cells

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2
Q

What is the inheritance pattern of G6PD deficiency?

A

X-linked recessive

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3
Q

What ethnicities is G6PD deficiency most commonly seen in?

A

African and Mediterranean

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4
Q

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

A

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

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5
Q

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

A

Heinz bodies and bite cells

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6
Q

Is thalassemia a macrocytic or microcytic anemia?

A

Microcytic

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7
Q

What ethnicity is alpha thalassemia commonly found in?

A

Southeast Asian and Chinese

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8
Q

What ethnicity is beta thalassemia most commonly found in?

A

African and Mediterranean

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9
Q

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

A
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
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10
Q

What chromosome is the alpha gene found on?

A

Chromosome 16

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11
Q

What is the inheritance pattern of sickle cell anemia?

A

Autosomal recessive

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12
Q

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

A

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

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13
Q

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

A

Acute chest syndrome

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14
Q

What is the definitive diagnostic study for sickle cell anemia?

A

Electrophoresis

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15
Q

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

A

Hydroxyurea

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16
Q

What is the mechanism of action of hydroxyurea?

A

Increases production of hemoglobin F and reduces production of hemoglobin S

17
Q

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

A

They have a dysfunctional spleen

18
Q

What anemias a megaloblastic anemias?

A

Vitamin B12 deficiency and folate deficiency anemias

19
Q

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

A

Strict veganism

20
Q

What is the most common cause of vitamin B12 deficiency?

A

Pernicious anemia

21
Q

What is pernicious anemia?

A

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

22
Q

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

A

Accumulation of methylmalonic acid causes degeneration of the spinal cord

23
Q

What type of anemia classically presents with koilonychia and pica?

A

IDA

24
Q

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

A

Thrombotic thrombocytopenic purpura

25
Q

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

A

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

26
Q

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

A

Hemolytic uremic syndrome.

27
Q

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

A

Vertebral fractures

28
Q

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

A

Multiple myeloma

29
Q

What protein is commonly seen in urine in multiple myeloma?

A

Bence Jones protein

30
Q

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

A

Punched out lesions

31
Q

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

A

Jaw.

32
Q

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

A

Rouleaux formations

33
Q

Hemophilia A results from deficiency of what clotting factor?

A

Factor VIII

34
Q

Hemophilia B results from lack of what clotting factor?

A

Factor IX

35
Q

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

A

Desmopressin acetate

36
Q

What factor enhances platelet aggregation and adhesion?

A

Von Willebrand factor.

37
Q

What is the inheritance pattern of hemophilias?

A

X-linked recessive