Intrinsic hemolytic anemia L.4-6

Question 1

Main characteristics of hemolytic anemias

Answer 1

Decreased RBC production
Increased RBC destruction or loss

= rate of destruction exceeds marrow ability to produce

Question 2

Name the 5 manifestations of hemolytic anemias

Answer 2

1. Physical: jaundice (due to increased bilirubin), splenomegaly
2. High bilirubin, low haptoglobin, free hemoglobin
3. Increased erythropoiesis = high reticulocytes
4. Immature RBC in peripheral blood
5. Hemosiderinuria (high iron)

Question 3

What is caused by a defective binding of spectrin to protein 4,1?

Answer 3

Hereditary spherocytosis

Question 4

What is the most common enzyme deficiency in the Hexose monophosphate shunt. And how is the most common inclusion body form?

Answer 4

The hemoglobin in RBC without G6PD enzyme is oxidized which precipitates and form the Heinz body.

Question 5

What can cause G6PD anemia?

Answer 5

Drugs (anti-malarial)
Fava beans
Chemicals

Question 6

What can happen to the blood picture after ingesting Fava beans for those with G6PD deficiency?

Answer 6

Hemoglobin decreases
High reticulocytes
Bite cells appear
Heinz bodies

Question 7

Principle of the G6PD Beutler fluorescent spot test?

Answer 7

Reduction of NADP to NADPH with formation of 6PG which fluoresces.

Question 8

Why does hemolytic anemia happen in pyruvate kinase deficiency anemia?

Answer 8

Decrease amount of ATP production which allows leakage of potassium out of the cell.

Question 9

What is the condition where cells are susceptible to mild acidity?

Answer 9

Paroxysmal nocturnal hemoglobinuria

Question 10

What can trigger PNH?

Answer 10

Nighfall

Exercise

Question 11

Blood picture for PNH

Answer 11

Increased reticulocytes
Low hemoglobin
Can result in low leukocytes, platelets

Question 12

What are the symptoms of acute blood loss?

Answer 12

Hypovolemic shock (shock due to loss of fluid)
Clammy skin with feeling cold
Confusion
Tachycardia, hypotension

Question 13

What is the overall blood picture for acute blood loss?

Answer 13

Beginning:
Slightly low RBC
N Hct
WBC and Pltmight increase due to emptying of the marginal pool.

Later:
Increased reticulocytes
hyperplasia of BM
low Hgb-Hct-RBC; N or high MCV
polychromasia and macrocytes (due to accelerated erythropoiesis)
No poikilocytes or inclusions due to very young cells

Question 14

What is the overall blood picture of aplastic anemia and what causes it?

Answer 14

Caused by failure of the BM which decreased the production of stem cell growth resulting in pancytopenia and hypocellularity.

Question 15

What are aplastic anemic patient prone to?

Answer 15

Infections (low WBC)

Bleeding (low RBC and PLT)

Question 16

What happens in Pure red cell aplasia?

Answer 16

In adults, there is a decrease in the production of cells in the RBC line (only affect RBC, WBC and PLT line are normal).
In children, they are unable to use up erythropoietin (Diamond-Blackfan).