Hematology - Anemias Review

Question 1

What does it mean to increase and decrease the RPI?

Answer 1

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

Question 2

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

Answer 2

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

Question 3

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

Answer 3

Iron deficiency

Question 4

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

Answer 4

Chronic disease

Question 5

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

Answer 5

Hemochromatosis

Question 6

What are the clinical signs of beta thalassemia major?

Answer 6

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

Question 7

What are the clinical signs of beta thalassemia major?

Answer 7

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

Question 8

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

Answer 8

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

Question 9

What is the enzyme affected in lead poisoning?

Answer 9

ALA dehydratase

Question 10

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

Answer 10

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

Question 11

What are the neurologic structures affected in cobalamin deficiency?

Answer 11

Spinocerebellar tract, lateral corticospinal tract, dorsal columm dysfunction.

Question 12

What are the causes of non megaloblastic anemia?

Answer 12

Chronic alcohol overuse, liver disease and Diamond Blackfan anemia

Question 13

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

Answer 13

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.

Question 14

Explain the decrease in iron in anemia of chronic disease.

Answer 14

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

Question 15

What are the intrinsic hemolytic anemias?

Answer 15

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

Question 16

What are the causes of extrinsic hemolytic anemias?

Answer 16

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

Question 17

What are the findings in intravascular hemolysis?

Answer 17

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

Question 18

What are the findings in extravascular hemolysis?

Answer 18

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

Question 19

Classify the intrinsic hemolytic anemias in intravascular or extravascular hemolysis.

Answer 19

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

Question 20

Name examples of drugs that cause hemolytic anemia.

Answer 20

Antibiotics (penicillins, cephalosporins)
NSAIDs
Immunotherapy
Chemotherapy

Question 21

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

Answer 21

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.

Question 22

Explain the mechanism of micro and macroangiopathic hemolytic anemia.

Answer 22

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.