Hemolytic Anemia

Question 1

What is another name for hemolytic anemia?

Answer 1

Survival Defect

Question 2

What is the RBC survival time in hemolytic anemia?

Answer 2

Less than 100 days

Question 3

Hemolytic Anemia

1. Classification (2)
   - Grouped by cause of premature RBC destruction

Answer 3

Intravascular

Extravascular

Question 4

Hemolytic Anemia

1. Clinical Symptoms

Answer 4

1. Jaundiced
2. New onset of pallor and anemia
3. Discoloration of the urine
4. Gallstones
5. Splenomegaly (preferential site of hemolysis)

Question 5

Hemolytic Anemia

1. Diagnostic Labs

Answer 5

1. Haptoglobin decreased –> hemolysis

***Haptoglobin are dump trucks***

2. LDH elevated in intra- and extravascular hemolysis
   - Due to bursting open of cells
3. Coombs test positive
4. Reticulocyte count elevated
5. Indirect (i.e. unconjugated) bilirubin elevated

***Causes jaundice***

6. Urine hemosiderin high –> intravascular hemolysis

Question 6

G6PD Deficiency (causes hemolytic anemia

1. Buzz Words

Answer 6

X-linked

Most Common enzymatic disorder

Fava Beans

Heinz Bodies

Bite Cells

Mnemonic: My X Girlfriend Bites Fava Beans with Heinz Ketchup

Question 7

Answer 7

Bite Cells

Question 8

Question 9

Answer 9

Heinz Bodies

Question 10

Pyruvate Kinase Deficiency

1. Just know that it is in the realm of hemolytic anemia and is less common than G6PD deficiency

Question 11

Sickle Cell Syndromes

1. Mutation

Answer 11

Mutation in the B-globulin in which Valine is substituted in place of Glutamine

***Both B-globulin chains affected –> Sickle Cell Anemia (SCA)

Question 12

What infections are patients with Sickle Cell Anemia more at risk for?

Answer 12

More at risk for infection by encapsulated organisms and Salmonella

(e.g. strep pneumoniae, meningococcal, H. influenzae, Hepatitis B, and Influenza vaccines)

Question 13

Hereditary Spherocytosis and Eliptocytosis

1. Mutation/Demographic
2. Effects
3. Complications
4. Dx/Tx

Answer 13

Autosomal dominant in Northern Europeans

Loss of membrane flexibility and chronic hemolysis

Complications –> Cholelithiasis (d/t bili stones) splenomegaly

Dx: Osmotic Fragility Test

Tx: Folate Supplementation, Genetic Counseling, Splenectomy

Question 14

Spherocytosis

Answer 14

Sphero/Eliptocytosis

Question 15

Paroxysmal Nocturnal Hemoglobinuria (PNH)

1. Mutation

2 Triad?

3. Treatment

Answer 15

Defective PIG-A gene

Triad –> Hemolysis, Pancytopenia, Risk of Venous Thrombosis

Treatment: Hemolysis w/ corticosteroids, anticoagulants for thrombolytic events, Eculizumab - ab to C5 terminal complement - decreases hemolysis and need for transfusion

Question 16

Autoimmune diseases will all usually respond to what treatment?

Answer 16

Treatment with steroids

Question 17

Autoimmune Hemolytic Anemia

1. Cause
2. Symptoms
3. Treatment

Answer 17

1. Caused by IgG or IgM antibodies
2. Clinically present with splenomegaly
3. Treatment: Corticosteroids

Question 18

If an attending ever asks you what could be the cause of something, always say what?

Answer 18

Drugs (drugs cause everything)

Question 19

Drug-Associated Hemolysis

Answer 19

1. Penicilins
2. Quinine, methyldopa, certain cephalosporin antibiotics are known culprits

Question 20

Functionally asplenic patients are at risk for what type of organisms?

Answer 20

Encapsulated (e.g. Strep pneumoniae)

and

Salmonella

Question 21

What is the most common enzymatic defect in RBCs?

Answer 21

G6PD deficiency

Question 22

Which 3 lab values and their respective changes would best reflect hemolytic anemia?

Answer 22

Indirect bilirubin elevated

LDH elevated

Reticulocyte count elevated

Question 23

Heinz Bodies and Bite Cells are seen in which Hemolytic Anemia process?

Answer 23

G6PD Deficiency

Mnemonic: My X Girlfriend Bites Fava Beans with Heinz Ketchup