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Medicine

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Hematology

Question
8 out of 15
 

All of the following can be associated with PNH Except: (AIPG 2009)



A Cerebral thrombosis

B Budd Chiari syndrome

C Pancytopenia

D Massive splenomegaly

Ans. D

Massive splenomegaly

Clinical Features of P.N.H.

1. Intravascular hemolysis

a. Most of the clinical features of P.N.H. can be attributed to intravascular hemolysis. The hemolysis is paroxysmal and usually occurs in the night.

b. During sleep at night, we all tend to Retain CO2 leading to mild respiratory acidosis the pH of the blood gets slightly reduced, low pH leads to activation of the complement

c. Activation of the complement in turn causes intravascular hemolysis, leukopenia, thrombocytopenia (Pancytopenia)

d. This is also the basis for Ham's acidified serum test which is used to diagnose P.N.H. When the blood is placed in acidic medium, it undergoes hemolysis.

e. Splenomegaly is not a feature.

2. Thrombosis

a. Platelets are decreased in P.N:H. But 'despite the presence of thrombocytopenia, PNH is associated with increase incidence of thrombosis.

b. Budd Chiari syndrome is one of the common clinical manifestations of thrombosis, it occurs due to hepatic vein thrombosis. Ascites with Acute hepatomegaly with pain abdomen, without previous liver disease

Hematology Flashcard List

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