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Paroxysmal Nocturnal hemoglobinuria (PNH)

  1. The disease in not inherited, it is an acquired disorder of hematopoiesis characterized by defect in protein of cell membrane that render the RBC susceptible to damage by serum complement. 
  2. Deficient membrane associated protein include decay- accelerating factor, the C8 binding protein that normally impede complement lysis at various steps. 
  3. Clinical manifestation
    1. Marrow failure,
    2. Chronic anemia with intravascular hemolysis.
    3. Nocturnal and morning hemoglobinuria is a classic finding.
    4. Thromboembolic complications lead to abdominal/ back and head pain. 


  1. Positive acid serum (Ham) test or sucrose lysis test.
  2. Hemosiderinuria.
  3. ↓ RBC Acetylcholinesterase
  4. Flow cytometry is more sensitive test.

Treatment- splenectomy is not indicatedQ. Treated by Prednisone

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