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USMLE Step 1 > Hematology - Anemias Review > Flashcards

Hematology - Anemias Review Flashcards

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USMLE® Step 1 flashcards
1
Q

What does it mean to increase and decrease the RPI?

A

Hight RPI (>3%) means a compensatory RBC production
Low RPI (<3%) indicates inadequate response to correct anemia

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2
Q

What does it mean to Mentzer index > 13 and < 13.

A

An index of > 13 suggest iron deficiency anemia
An index of < 13 suggest thalassemia

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3
Q

Which disease has low iron, high TIBC, low Ferritin and low transferrin saturation?

A

Iron deficiency

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4
Q

Which disease has low iron, low TIBC, high Ferritin and low transferrin saturation?

A

Chronic disease

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5
Q

Which disease has high iron, low TIBC, high ferritin and high transferrin saturation?

A

Hemochromatosis

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6
Q

What are the clinical signs of beta thalassemia major?

A

Severe microcytic anemia with target cells
Anisopoikilocytosis
Increase risk of hemocromatosis due increase requiring blood transfusions
Marrow expansion
Skeletal deformidades
Extramedullary hematopoiesis

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7
Q

What are the clinical signs of beta thalassemia major?

A

Severe microcytic anemia with target cells
Anisopoikilocytosis
Increase risk of hemocromatosis due increase requiring blood transfusions
Marrow expansion
Skeletal deformidades
Extramedullary hematopoiesis

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8
Q

Explain the sign “crew cut” on skull x-ray

A

It corresponds to the increase in extramedullary hematopoiesis, and can be seen in patients with beta thalassemia.

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9
Q

What is the enzyme affected in lead poisoning?

A

ALA dehydratase

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10
Q

What is the enzyme affected in sideroblastic anemia? What is your relationship with tuberculosi treatment?

A

ALA synthase
Decrease vitamin B6. Deficiency in this vitamin is commonly seen in patients taking isoniazid for the treatment of tuberculosis.

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11
Q

What are the neurologic structures affected in cobalamin deficiency?

A

Spinocerebellar tract, lateral corticospinal tract, dorsal columm dysfunction.

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12
Q

What are the causes of non megaloblastic anemia?

A

Chronic alcohol overuse, liver disease and Diamond Blackfan anemia

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13
Q

What is the problem with Diamond Blackfan anemia? What is the difference with Fanconi anemia?

A

Diamond Blackfan anemia is due to intrinsic defect in erythroid progenitor cells. This is a congenital form of pure red cell aplasia, different from Fanconi anemia, which causes pancytopenia.

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14
Q

Explain the decrease in iron in anemia of chronic disease.

A

Infammation increase IL-6 -> increase hepcidin-> decrease release of iron from macrophages and decrease iron absorption from gut.

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15
Q

What are the intrinsic hemolytic anemias?

A

Hereditary spherocytosis
Paroxysmal nocturnal hemoglobinuria
G6PD deficiency
Pyruvate kinase deficiency
Sickle cell anemia
HbC disease

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16
Q

What are the causes of extrinsic hemolytic anemias?

A

Autoimmune hemolytic anemia
Drug induced hemolytic anemia
Microangiopathic hemolytic anemia
Macroangiopathic hemolytic anemia
Infection

17
Q

What are the findings in intravascular hemolysis?

A

Low haptoglobin, high schistocytes on blood smear, hemoglobinuria, hemosiderinuria and urobilinogeb in urine. Increase DHL.

18
Q

What are the findings in extravascular hemolysis?

A

Splenomegaly, spherocytes in peripheral smear, NO hemoglobinuria/hemosiderinuria. Can present with urobilinogen in urine.

19
Q

Classify the intrinsic hemolytic anemias in intravascular or extravascular hemolysis.

A

Hereditary spherocytosis -> extravascular hemolysis
Paroxysmal nocturnal hemoglobinuria -> intravascular hemolysis
G6PD deficiency -> extravascular and intravascular hemolysis
Pyruvate kinase deficiency -> extravascular hemolysis
Sickle cell anemia -> intravascular and extravascular hemolysis.
HbC disease extravascular hemolysis

20
Q

Name examples of drugs that cause hemolytic anemia.

A

Antibiotics (penicillins, cephalosporins)
NSAIDs
Immunotherapy
Chemotherapy

21
Q

There are two types of Autoimmune Hemolytic Anemia (AIHA), explain.

A

Warm AIHA - Chronic anemia in which primarily IgG causes extravascular hemolysis.

Cold AIHA - Acute anemia in which primarily IgM + complement cause RBC agglutination and extravascular hemolysis upon exposure to cold.

22
Q

Explain the mechanism of micro and macroangiopathic hemolytic anemia.

A

Microangiopathic -> RBC are damage when passing through obstructed or narrowed vessels. Seen in DIC, TTP/HUS, SLE, HELLP syndrome, hypertensive emergency.

Macroangiopathic -> results from shear stress caused by turbulent blood flow or occurs in patients with prosthetic heart valves and aortic stenosis.

USMLE Step 1 (3 decks)

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