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Paroxysmal Nocturnal Hemoglobinuria



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Paroxysmal Nocturnal Hemoglobinuria

Slide 2

December 2010

Paroxysmal nocturnal hemoglobinuria is a rare form of acquired hemolytic anemia. The red blood cells get destroyed by complement-mediated lysis which can lead to hemoglobinuria, renal failure and pulmonary hypertension, as well as symptoms such as dysphagia and lethargy. The most serious complication of PNH is venous thrombosis; it often presents in unusual sites such as hepatic, splenic and mesenteric veins. Finally, the disease is characterized by an underlying bone marrow failure presented as severe aplastic anemia in extreme cases. From this variety of symptoms, as well as from the laboratory studies, it is now understood that PNH is a hematopoietic stem cell disorder affecting all blood cell lineages.

Paroxysmal Nocturnal Hemoglobinuria

 


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