Paroxysmal Nocturnal Hemoglobinuria
Paroxysmal Nocturnal Hemoglobinuria is a very rare disease occurs in 1 every million worldwide. It hard to differentiate its symptoms from other overlapping conditions, and takes long time to be properly diagnosed.It is mainly caused by mutation in genes that code anchoring proteins on different blood cells types. The different symptoms mainly depends on intravascular hemolysis. The management aimed to correct the anemia and to prevent life-threatening conditions as thrombi and intravascular clotting
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Paroxysmal Nocturnal Hemoglobinuria originates from mutant genes in stem cells of bone marrow that differentiate to different types of blood cells.Hemolytic anemia, bone marrow failure and thrombophilia is Clinical triad typical for PNH. The management depend on patient case