EXAM #1: HEMOLYTIC ANEMIA

Question 1

What is the Hb definition of anemia in males? Females?

Answer 1

Males= less than 13.5 g/dL

Females= less than 12.5 g/dL

Question 2

What is the base definition of “hemolytic” anemia?

Answer 2

Destruction of RBCs that exceeds the bone marrows ability to produce RBCs

Question 3

What are the two major subdivisions of hemolytic anemia?

Answer 3

Intravascular

Extravascular

Question 4

Clinically, what do most patients with hemolytic anemia present with?

Answer 4

Jaundice

Unconjugated/ indirect bilirubin

Question 5

What are the clinical signs that are specific to hemolytic anemia?

Answer 5

• Jaundice
• New onset pallor and dyspnea
• Discoloration of urine
• Gallstones
• Splenomegaly

Question 6

What are the labs that are diagnostic to hemolytic anemia?

Answer 6

1) Decreased haptoglobin
2) Lactate dehydrogenase (LDH) elevated
3) Elevated retic.
4) Elevated indirect/unconjugated bilirubin
5) High urine hemosiderin

“Haptoglobin are dump-trucks”

Question 7

Why is Haptoglobin decreased in hemolytic anemia?

Answer 7

• Haptoglobin binds free Hb in the blood and transports it

- Haptoglobin is LOW b/c destruction of RBCs leaves free Hb in the serum

Question 8

Why is LDH elevated in hemolytic anemia?

Answer 8

• LDH is an enzyme in RBCs

- Rupture of cells releases LDH into the serum

Question 9

What is a positive Coombs test indicative of?

Answer 9

Antibody or complement mediated hemolysis

Question 10

What does an elevated corrected retic. count mean?

Answer 10

RBC production is working

Question 11

List the labs that need to be drawn for a suspected hemolytic anemia.

Answer 11

Haptoglobin 
LDH 
Coombs test 
Indirect bilirubin
Retic count
Urine hemosiderin

Question 12

What is the most common disorder that causes hemolytic anemia?

Answer 12

G6PD

Question 13

List the characteristics of G6PD.

Answer 13

• X-Linked
• African and Mediterranean
• Unable to regenerate reduced glutathioine b/c of a lack of NADPH from HMP shunt

Question 14

What are the abnormal cell types that are specific to G6PD?

Answer 14

Heinz bodies

Bite cells

Question 15

List the characteristics of Pyruvate Kinase Deficiency.

Answer 15

• Less common than G6PD but causes MORE SEVERE hemolytic anemia
• Often presents in newborns w/ neonatal jaundice

Hemolytic crisis without exposure to oxidative agents

Question 16

What is the mutation that causes Sickle Cell Syndrome?

Answer 16

Mutation of Beta-globin in which valine replaces glutamine

Question 17

What is the difference between Sickle Cell Trait and Disease?

Answer 17

Trait= 1x Beta-globin affected

Disease= BOTH

Question 18

What type of Hb is seen in Sickle Cell Anemia?

Answer 18

HbS

Question 19

What are the clinical manifestations of Sickle Cell Anemia?

Answer 19

1) Tissue infarction
2) Recurrent renal infarcts leading to isosthenuria
3) Recurrent splenic infarcts leading to asplenia

Question 20

What infections are patients with Sickle Cell Disease at risk for?

Answer 20

Asplenia= susceptibility to encapsulated microorganisms:

1) S. pneumonia
2) Megingococcal
3) H. influenzae
4) Hep B
5) Influenza
6) Salmonella

Question 21

What is Hereditary Spherocytosis?

Answer 21

Autosomal dominant disease effecting the RBC cytoskeletal proteins, resulting in spherical RBCs that undergo extravascular hemolysis

Question 22

What are the complications of Hereditary Spherocytosis?

Answer 22

1) Cholelithiasis

2) Splenomegaly

Question 23

How is Hereditary Spherocytosis diagnosed?

Answer 23

Osmotic Fragility Test

Question 24

How is Hereditary Spherocytosis treated?

Answer 24

• Folate supplementation

- Splenectomy

Question 25

What is the typical treatment for autoimmune hemolytic anemia?

Answer 25

Corticosteroids

Question 26

What drugs are associated with hemolysis?

Answer 26

• PCN
• Quinine
• Methyldopa
• Cephalosporin antibiotics