HEMOLYTIC ANEMIAS

Question 1

Defects in membrane skeletal proteins; membrane skeletal protein abnormalities cause RBC to lose unsupported lipid membrane

Answer 1

Hereditary Spherocytosis

Question 2

laboratory findings in hereditary spherocytosis

Answer 2

• spherocytes
• MCHC >37 g/dL
• High osmotic pressure
• High serum bilirubin

Question 3

membrane defect due to abnormal permeability both to sodium and potassium; causes erythrocyte swelling

Answer 3

hereditary stomatocytosis

Question 4

Membrane defects caused by polarization of cholesterol at the ends of the cell rather than around pallor area

Answer 4

Hereditary elliptocytosis (ovalocytosis)

Question 5

Increased cholesterol:lecithin ratio in the membrane due to abnormal lipid concentrations; absence of serum beta-lipoprotein needed for transport

Answer 5

Hereditary acanthocytosis (abetalipoproteinemia)

Question 6

most common enzyme deficient in HMP

Answer 6

Glucose-6-phosphate dehydrogenase (G6PD)

Question 7

most common enzyme deficient in EMP

Answer 7

Pyruvate Kinase (PK)

Question 8

Acquired defect in which RBC membrane has an increased sensitivity for complement binding as compared to normal RBCs

Answer 8

Paroxysmal Nocturnal Hemoglobinuria (PNH)

Question 9

A hemolytic anemia that involves IgG antibodies with P specificity fixes complement to RBCs in the cold (<20C); complement-coated RBC lyes when warmed at 37C

Answer 9

Paroxysmal Cold Hemoglobinuria

Question 10

What IgG antibody is present in PCH?

Answer 10

Donath-Landsteiner Antibody

Question 11

Hemolytic anemia where RBCs are coated with IgM and complement at temperatures <37C

Answer 11

Cold Autoimmune Hemolytic Anemia

Question 12

Anti-I is produced by what organism?

Answer 12

Mycoplasma pneumoniae

Question 13

Anti-i is produced by what organism?

Answer 13

Epstein-Barr Virus (EBV)

Question 14

The recipient has antibodies to antigens on donor RBCs; donor cells are destroyed

Answer 14

Hemolytic transfusion reaction

Question 15

DIsease due to different group antigens on the parents of a newborn

Answer 15

Hemolytic disease of the newborn (HDN)

Question 16

Systemic clotting is initiated by the activation of the coagulation cascade due to toxins or conditions that trigger the release of coagulants. Multiple organ failure occurs due to clotting; fibrin is deposited in small vessels, causing RBCs to FRAGMENYT (schistocytes)

Answer 16

Disseminated Intravascular Coagulation (DIC)

Question 17

Occurs most often in children following gastrointestinal infection (e. g. Escherichia coli); clots form causing renal damage;

Answer 17

Hemolytic Uremic Syndrome (HUS)

Question 18

Triad of HUS

Answer 18

1. MAHA (schistocytes)
2. Renal damage
3. Thrombocytopenia

Question 19

typical HUS:

Answer 19

Shiga toxin-associated HUS, or Stx-HUS

Question 20

bacteria that can produce Shiga toxin

Answer 20

• E. coli O157H7 (common)
• Shigella

Question 21

Anything that can damage/disturb endothelial cells will lead to _______

A. thrombosis
B. thrombocytopenia

Answer 21

A, Thrombosis (clotting)

Question 22

An enzyme that is responsible for the breakdown of large von Willebrand factor multimers;
a deficient of this enzyme can lead to ______

Answer 22

• ADAMTS13
• thrombotic thrombocytopenic purpura (TTP)

Question 23

Pentad of TTP

Answer 23

1. MAHA (schistocytes)
2. Renal damage
3. Thrombocytopenia
4. Fever
5. CNS impairment