paroxysmal nocturnal hemoglobinurea

Question 1

site of hemolysis in paroxysmal nocturnal hemoglobinurea ?

Answer 1

intravascular

Question 2

etiology Paroxysmal nocturnal haemoglobinuria

Answer 2

caused by increased sensitivity of cell membranes to complement (see below) due to a lack of glycoprotein glycosyl-phosphatidylinositol (GPI).

Question 3

complications with paroxysmal nocturnal hemoglobinuria are

Answer 3

clots - venous thrombosis - budd chiari syndome

Question 4

clinical features of paroxysmal nocturnal hemoglobinurea ?

Answer 4

hematurea
haemglobinurea - dark coloured urine in the morning

Question 5

blood investigations for paroxysmal nocturnal hemoglobinuria?

Answer 5

hemolytic anemia
pancytopenia may be seen- because all the cells might be sensitive to the compliment
= aplastic anemia

Question 6

diagnosis of paroxysmal nocturnal hemglobinurea ?

Answer 6

flow cytometry detcting low levels of cd59 and cd55

Ham’s test: acid-induced haemolysis (normal red cells would not)

Question 7

PNH can be treated with

Answer 7

eculizumab