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Medicine

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Hematology

Question
12 out of 15
 

Which of the following tests is gold standard to diagnose a case of PNH? (NEET 2013)



A HAMS test
B Sucrose lysin test

C Flow cytometry
D Bone marrow

Ans. C

Flow cytometry

Diagnosis of PNH

Tests: 1. HAMS test 2. Sucrose lysin test 3. Flow cytometry

1. Flow cytometry is the Gold standard in diagnosis, Analysis GPI linked proteins CD 59, DAF

2. Bone Marrow in P.N.H.

a. Normoblastic hyperplasia is the characteristic finding in P.N.H. (Pancytopenia with normoblastic hypercellular BM)

b. Important Points:: Normoblastic hyperplasia is a feature of PNH. At some stage of disease BM may become hypocellular or even aplastic

3. Leucocyte Alkaline Phosphatase in P.N.H.

The level of neutrophil alkaline phosphatase is decreased in P.N.H.

Tests: a. Hb b. MCV

Extra Edge:

1. HAM test is based upon susceptibility of RBC’s to complement mediated lysis in patients with PNH. In acidified serum complement is activated by the alternate pathway.

2. The fluorescein-labeled proaerolysin (FLAER) test is being used more frequently to diagnose PNH. FLAER binds selectively to the glycophosphatidylinositol anchor and is more accurate in demonstrating a deficit than flow cytometry for CD59 or CD55.

Complication: AML can occur in 10% cases

Natural History of PNH

1. Aplastic anemia can occur

2. AML can occur

3. Spontaneous recover can occur but it rare

Hematology Flashcard List

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