G6PD AND acquired HA

Question 1

Type of congenital hemolytic anemia causes

Answer 1

Membranes defect
enzymes defect
globin defect

Question 2

Type of acquired causes of Hemolytic anemia

Answer 2

Immune

non-immune

Question 3

Do red cells need continuous supply of energy for maintenance of membrane flexibility and cell shape

Answer 3

Yes

Question 4

What is the main source of energy of red cell and why

Answer 4

Glucose is the main source of energy because red cells lack mitochondria

Question 5

What is the function of G6PD in the glucose pathway

Answer 5

Reduce NADPH which is essential for reduction of glutathione which forms antioxidants essential for rbc

Question 6

Is G6PD deficiency sex linked

Answer 6

Yes

Question 7

What are the two phenotype of G6PD

Answer 7

G6PD A+. If normal

G6PD A- if defect( Africa )

Question 8

Main race is affected by G6PD defects

Answer 8

West Africans
Mediterranean’s
middle east
south east Asia

Question 9

Is G6PD deficiency symptomatic or mostly asymptomatic

Answer 9

Asymptomatic

Question 10

Is there neonatal jaundice

Answer 10

Yes

Question 11

Why is there hemolytic anemia in G6PD deficiency

Answer 11

Due to oxidant stress especially when taking drugs when eating fava beans or during infections

Question 12

How is the Enzyme deficiency Tested

Answer 12

Methaemoglobin reduction test

Direct enzyme Assay on red cells

Question 13

What appears on a blood film of G6PD deficiency Patient

Answer 13

Fragmented cell
contracted cell
bites cells
blister cells

Question 14

How is the blood counts in G6PD deficiency

Answer 14

Normal between crisis

Question 15

When does Hemolysis and anemia start in a G6PD deficiency crisis

Answer 15

Hemolysis begins 1 to 3 days after exposure

anemia begins about 7 to 10 days after exposure

Question 16

Is there intravascular hemolysis features in G6PD deficiency

Answer 16

Yes

Question 17

Does pyruvate kinase deficiency affect equally all ethnic origins

Answer 17

Yes

Question 18

Does pyruvate kinase deficiency protect in plasmodium falciparum infection

Answer 18

Yes

Question 19

Can you do splenectomy in pyruvate kinase deficiency

Answer 19

In some patients

Question 20

Is there a chronic hemolytic anemia in Pyruvate kinase efficiency

Answer 20

Yes

Question 21

How do you test for a acquired hemolytic anemia is caused by immune reaction

Answer 21

Coombs test

Question 22

What are the different type of auto immune acquired hemolytic anemia’s

Answer 22

Either warm or cold based on if the antibody react more strongly with red cells at 37° or 4°C

Question 23

What’s proteins coats the red cell in a warm immune hemolytic anemia

Answer 23

Immunoglobin G alone or with compliment

Question 24

What happens when The coated membrane of the cells in warm immune hemolytic anemia get Partially phagocytoses in the spleen

Answer 24

Become spherocyte

Question 25

Causes of warm reactive antibodies

Answer 25

``` Idiopathic SLE some drugs chronic lymphocytic leukemia lymphoma ```

Question 26

Causes of cold reactive antibodies

Answer 26

Idiopathic mycoplasma pneumonia infection infectious mononucleosis Lymphomas

Question 27

Immunoglobulin type in cold reactive antibodies

Answer 27

IgM

Question 28

Is there chronic anemia accentuated by cold in cold autoimmune hemolytic anemia

Answer 28

Yes

Question 29

2 types of alloimmune hemolytic anemias

Answer 29

Hemolytic transfusion rxn | Hemolytic disease of the newborn

Question 30

Types of drugs that can cause drug induced hapten type HA

Answer 30

Penicillin | Rarely cephalosporin

Question 31

Drug induced non immunological HA mechanism

Answer 31

Drug alters RBC membrane which adsorb IgG and complement on the surface May cause hemolytic anemia

Question 32

Drugs that can cause drug induced non immunological HA

Answer 32

Cephalosporin

Question 33

Causes of acquire non immune HA

Answer 33

Mechanical trauma to red cells like aortic valve prostheses ,severe aortic valve disease, microangiopathic HA, hemolytic uremic syndrome, thrombotic thrombocytopenia purpurine, metastatic malignancy , malignant hypertension, DIC, March hemoglobinuria Burns Infections like malaria , clostridium per fringe s Drugs Hypersplenism

Question 34

What is paroxysmal nocturnal hemoglobinuria

Answer 34

Red cell with defective membrane lack several protein which were supposed to inhibit complement RBC very sensitive to complement creating chronic Intravascular hemolysis

Question 35

Test for PNH

Answer 35

Acidified serum test - Ham’s test