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Hematology > G6PD AND acquired HA > Flashcards

G6PD AND acquired HA Flashcards

1
Q

Type of congenital hemolytic anemia causes

A

Membranes defect
enzymes defect
globin defect

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2
Q

Type of acquired causes of Hemolytic anemia

A

Immune

non-immune

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3
Q

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

A

Yes

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4
Q

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

A

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

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5
Q

What is the function of G6PD in the glucose pathway

A

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

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6
Q

Is G6PD deficiency sex linked

A

Yes

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7
Q

What are the two phenotype of G6PD

A

G6PD A+. If normal

G6PD A- if defect( Africa )

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8
Q

Main race is affected by G6PD defects

A

West Africans
Mediterranean’s
middle east
south east Asia

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9
Q

Is G6PD deficiency symptomatic or mostly asymptomatic

A

Asymptomatic

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10
Q

Is there neonatal jaundice

A

Yes

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11
Q

Why is there hemolytic anemia in G6PD deficiency

A

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

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12
Q

How is the Enzyme deficiency Tested

A

Methaemoglobin reduction test

Direct enzyme Assay on red cells

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13
Q

What appears on a blood film of G6PD deficiency Patient

A

Fragmented cell
contracted cell
bites cells
blister cells

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14
Q

How is the blood counts in G6PD deficiency

A

Normal between crisis

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15
Q

When does Hemolysis and anemia start in a G6PD deficiency crisis

A

Hemolysis begins 1 to 3 days after exposure

anemia begins about 7 to 10 days after exposure

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16
Q

Is there intravascular hemolysis features in G6PD deficiency

A

Yes

17
Q

Does pyruvate kinase deficiency affect equally all ethnic origins

A

Yes

18
Q

Does pyruvate kinase deficiency protect in plasmodium falciparum infection

A

Yes

19
Q

Can you do splenectomy in pyruvate kinase deficiency

A

In some patients

20
Q

Is there a chronic hemolytic anemia in Pyruvate kinase efficiency

A

Yes

21
Q

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

A

Coombs test

22
Q

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

A

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

23
Q

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

A

Immunoglobin G alone or with compliment

24
Q

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

A

Become spherocyte

25
Q

Causes of warm reactive antibodies

A 
Idiopathic 
SLE 
some drugs 
chronic lymphocytic leukemia
 lymphoma
26
Q

Causes of cold reactive antibodies

A

Idiopathic
mycoplasma pneumonia infection
infectious mononucleosis
Lymphomas

27
Q

Immunoglobulin type in cold reactive antibodies

A

IgM

28
Q

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

A

Yes

29
Q

2 types of alloimmune hemolytic anemias

A

Hemolytic transfusion rxn

Hemolytic disease of the newborn

30
Q

Types of drugs that can cause drug induced hapten type HA

A

Penicillin

Rarely cephalosporin

31
Q

Drug induced non immunological HA mechanism

A

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

32
Q

Drugs that can cause drug induced non immunological HA

A

Cephalosporin

33
Q

Causes of acquire non immune HA

A

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

34
Q

What is paroxysmal nocturnal hemoglobinuria

A

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

35
Q

Test for PNH

A

Acidified serum test - Ham’s test

Hematology (24 decks)

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