G6PD AND acquired HA Flashcards
Type of congenital hemolytic anemia causes
Membranes defect
enzymes defect
globin defect
Type of acquired causes of Hemolytic anemia
Immune
non-immune
Do red cells need continuous supply of energy for maintenance of membrane flexibility and cell shape
Yes
What is the main source of energy of red cell and why
Glucose is the main source of energy because red cells lack mitochondria
What is the function of G6PD in the glucose pathway
Reduce NADPH which is essential for reduction of glutathione which forms antioxidants essential for rbc
Is G6PD deficiency sex linked
Yes
What are the two phenotype of G6PD
G6PD A+. If normal
G6PD A- if defect( Africa )
Main race is affected by G6PD defects
West Africans
Mediterranean’s
middle east
south east Asia
Is G6PD deficiency symptomatic or mostly asymptomatic
Asymptomatic
Is there neonatal jaundice
Yes
Why is there hemolytic anemia in G6PD deficiency
Due to oxidant stress especially when taking drugs when eating fava beans or during infections
How is the Enzyme deficiency Tested
Methaemoglobin reduction test
Direct enzyme Assay on red cells
What appears on a blood film of G6PD deficiency Patient
Fragmented cell
contracted cell
bites cells
blister cells
How is the blood counts in G6PD deficiency
Normal between crisis
When does Hemolysis and anemia start in a G6PD deficiency crisis
Hemolysis begins 1 to 3 days after exposure
anemia begins about 7 to 10 days after exposure
Is there intravascular hemolysis features in G6PD deficiency
Yes
Does pyruvate kinase deficiency affect equally all ethnic origins
Yes
Does pyruvate kinase deficiency protect in plasmodium falciparum infection
Yes
Can you do splenectomy in pyruvate kinase deficiency
In some patients
Is there a chronic hemolytic anemia in Pyruvate kinase efficiency
Yes
How do you test for a acquired hemolytic anemia is caused by immune reaction
Coombs test
What are the different type of auto immune acquired hemolytic anemia’s
Either warm or cold based on if the antibody react more strongly with red cells at 37° or 4°C
What’s proteins coats the red cell in a warm immune hemolytic anemia
Immunoglobin G alone or with compliment
What happens when The coated membrane of the cells in warm immune hemolytic anemia get Partially phagocytoses in the spleen
Become spherocyte
Causes of warm reactive antibodies
Idiopathic SLE some drugs chronic lymphocytic leukemia lymphoma
Causes of cold reactive antibodies
Idiopathic
mycoplasma pneumonia infection
infectious mononucleosis
Lymphomas
Immunoglobulin type in cold reactive antibodies
IgM
Is there chronic anemia accentuated by cold in cold autoimmune hemolytic anemia
Yes
2 types of alloimmune hemolytic anemias
Hemolytic transfusion rxn
Hemolytic disease of the newborn
Types of drugs that can cause drug induced hapten type HA
Penicillin
Rarely cephalosporin
Drug induced non immunological HA mechanism
Drug alters RBC membrane which adsorb IgG and complement on the surface
May cause hemolytic anemia
Drugs that can cause drug induced non immunological HA
Cephalosporin
Causes of acquire non immune HA
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
What is paroxysmal nocturnal hemoglobinuria
Red cell with defective membrane lack several protein which were supposed to inhibit complement
RBC very sensitive to complement creating chronic Intravascular hemolysis
Test for PNH
Acidified serum test - Ham’s test
