Modules 4 and 5: Normocytic and hemolytic anemias

Question 1

Pancytopenia

Answer 1

Decreased numbers of all cellular elements in the blood

Question 2

Aplastic anemia

Answer 2

A group of disorders characterized by depletion of cellular elements and fatty replacement of the bone marrow
A failure of marrow blast cells and stem cells to differentiate or proliferate in the presence of adequate nutrition and stimulus
Pancytopenia (blood) and hypoplasia (marrow)

Question 3

Example of an inherited aplastic anemia

Answer 3

Fanconi’s anemia

Question 4

Fanconi’s anemia

Answer 4

Autosomal recessive disease
Multiple chromosomal defects
Developmental abnormalities (physical and mental)
Hypoplastic marrow and pancytopenia
Usually fatal before age 8

Question 5

Two groups of acquired aplastic anemia

Answer 5

Primary (idiopathic, cause not apparent) and secondary aplastic anemias (due to damage of blast cells or stem cells by a variety of identified physical and chemical agents)

Question 6

Chemical agents that often produce hypoplasia

Answer 6

Benzene
Trinitrotoluene (TNT)
Arsenic
Insecticides
Weed killers

Question 7

Drugs that often produce hypoplasia

Answer 7

Antimicrobials (chloramphenicol, penicillin, tetracycline, streptomycin, sulfonamides, amphotercin) and analgesics (phenylbutazone, aspirin)

Question 8

The most common congenital (hereditary) form of aplastic anemia

Answer 8

Fanconi’s anemia

Question 9

Secondary causes of aplastic anemia

Answer 9

Chemical agents, drugs, insecticides, ionizing radiation, infections

Question 10

Appearance of the bone marrow in aplastic anemia

Answer 10

Hypoplasia

Question 11

Post-hemorrhagic anemia

Answer 11

A normochromic, normocytic anemia caused by acute blood loss

Question 12

3 phases of post-hemorrhagic anemia

Answer 12

1. Up to 24hrs after blood loss; little evidence of anemia
2. 24-72hrs after blood loss; normochromic normocytic anemia, Hb, RBC and Hct decrease
3. More than 72hrs after blood loss; Normochromic regenerative anemia; reticulocytosis (increase in immature RBCs)

Question 13

A brown coloured plasma from an anemic patient is suggestive of:

Answer 13

Intravascular hemolysis

Question 14

Typical findings in severe hemolytic anemias

Answer 14

Polychromasia, normoblastemia, spherocytosis, methemalbuminemia

Question 15

The antibody formed in paroxysmal cold hemoglobinuria

Answer 15

A cold IgG

Question 16

Cause of Raynaud’s phenomenon

Answer 16

Cold IgM autoantibodies

Question 17

Another name for cold IgM autoantibodies

Answer 17

Cold agglutinins

Question 18

Defect in hereditary elliptocytosis

Answer 18

Deficiency or abnormality of RBC membrane cytoskeletal proteins

Question 19

Defect in hereditary stomatocytosis

Answer 19

Increase in passive permeability of the cell membrane to Na and K which causes a net swelling of the cell (gain of Na and loss of K)

Question 20

With what kind of stain would we see Heinz bodies in RBCs?

Answer 20

Supra vital stain

Question 21

Sickle cell trait is on which chromosome?

Answer 21

Chromosome 11

Question 22

Sickle cell trait is associated with resistance to which organism?

Answer 22

Plasmodium falciparum

Question 23

Defect in hereditary spherocytosis

Answer 23

One or more of the RBC membrane proteins (spectrin, ankyrin, protein 4.2 and/or band 3) are abnormal or deficient, resulting in a decreased surface area to volume ratio and spheroidal RBCs that are rigid and not as deformable, thus their survival in the spleen is decreased

Question 24

Test used to differentiate hereditary spherocytosis from autoimmune hemolytic disease

Answer 24

DAT - in hereditary spherocytosis, the DAT is negative

Question 25

Most useful confirmatory test for hereditary spherocytosis

Answer 25

Osmotic fragility test (characteristically increased)

Question 26

Defect in hereditary acanthocytosis

Answer 26

Enzyme deficiency that results in abetalipoproteinemia (absence of beta-lipoprotein in the blood plasma and in cell membranes of RBCs and other body cells), resulting in an increased sphingomelin/lecithin ratio in the cell membrane and the formation of acanthocytes

Question 27

G-6-PD deficiency

Answer 27

Decreased G6PD causing decreased NADPH causing increased amount of glutathione resulting in increased methemoglobin (oxidized hemoglobin) due to oxidizing agents like food, medication, infections

Question 28

Pyruvate kinase deficiency

Answer 28

Decreased ATP production and therefore hemolysis due to failure of active transport and other ATP dependent reactions

Question 29

Polypeptide formula for HbS

Answer 29

Alpha2 beta2 6GLU->VAL (indicating the substitution of valine for glutamate in the 6th position of the beta chains of globin

Question 30

Hemoglobin variant that produces “Washington monument” tetrahedral crystals in RBCs

Answer 30

Hb C