Hematology

Question 1

What are the two types of inherited bone marrow failure syndromes?

Answer 1

Fanconi anemia & Diamond-Blackfan anemia (DBA)

Question 2

Which inherited bone marrow failure is autosomal recessive and presents with physical abnormalities?

Answer 2

Fanconi anemia

Question 3

Which inherited bone marrow failure syndrome is autosomal dominant?

Answer 3

Diamond-Blackfan anemia (DBA)

Question 4

What is the gold standard diagnostic test for Aplastic Anemia?

Answer 4

Bone marrow biopsy

Question 5

What are the peripheral blood smear & reticulocyte findings of Aplastic Anemia?

Answer 5

Normochromic, Normocytic; Low reticulocytes

Question 6

What does a bone marrow biopsy of Aplastic Anemia show?

Answer 6

Hypocellular, >70% yellow marrow

Question 7

What is the only cure for Aplastic Anemia?

Answer 7

Stem cell transplant (bone marrow, peripheral blood, cord blood)

Question 8

Treatments used for Aplastic Anemia?

Answer 8

Immunosuppressives (Corticosteroids, Cyclosporine, Anti-thymocyte globulin, Tacrolimus); Bone marrow stimulators

Question 9

Which type of anemia is commonly associated with Parvo B19?

Answer 9

Pure red cell aplasia

Question 10

Normocytic anemia w/ low reticulocytes, low RBCs, normal WBC & platelets

Answer 10

Pure red cell aplasia

Question 11

What does the bone marrow in pure red cell aplasia show?

Answer 11

Giant proerythroblasts

Question 12

3 causes of intravascular hemolysis

Answer 12

1. Activation of complement on RBC membrane
2. Physical or mechanical trauma to RBC
3. Toxic microenvironment

Question 13

3 causes of extravascular hemolysis

Answer 13

1. Inherited RBC defects
2. Acquired RBC defects
3. Immune-mediated hemolytic anemia

Question 14

Heme is converted through a series of steps to ________

Answer 14

bilirubin

Question 15

What count is nearly always elevated in patients w/ hemolysis?

Answer 15

Reticulocyte count (unless there is marrow suppression, such as by folic acid or iron deficiency)

Question 16

Caused by auto-antibodies to membranes of red blood cells

Answer 16

Auto-Immune Hemolytic Anemia

Question 17

MC AIHA

Answer 17

Warm antibody hemolytic anemia

Question 18

IgG mediated hemolytic anemias

Answer 18

Warm antibody hemolytic anemia & paroxysmal cold hemoglobinuria

Question 19

IgM mediated hemolytic anemia

Answer 19

Cold agglutinin disease

Question 20

IgG antibody binds to the erythrocyte at cold temperatures, setting the stage for complement-mediated hemolysis when the blood is back at a warm temperature

Answer 20

Cold agglutinin disease

Question 21

test used in AIHA evaluation

Answer 21

Coombs Test

Question 22

Which cold agglutinin disease (CAD) is due to infection (virus or mycoplasma), autoimmune disease, or lymphoid malignancy (B-cell or plasma)?

Answer 22

Secondary CAD

Question 23

MCC of AIHA in children

Answer 23

Paroxysmal cold hemoglobinuria (PCH)

Question 24

Acquired AIHA, due to autoantibody to P antigen on RBC, that fixes complement in the cold, but only activates after it rewarms

Answer 24

Paroxysmal cold hemoglobinuria (PCH)

Question 25

Acquired hemolytic disorder due to a mutation in a hematopoietic stem cell

Answer 25

Paroxysmal Nocturnal Hemoglobinuria (PNH)

Question 26

• RBCs are missing GPI-linked complement inhibitors
• Susceptible to complement-mediated intravascular hemolysis but NOT antibody mediated

Answer 26

Paroxysmal Nocturnal Hemoglobinuria