Tuesday 18 February 2014

Paroxysmal Nocturnal Hemoglobinuria

Paroxysmal nocturnal hemoglobinuria

Formerly Marchiafava–Micheli disease is an occasional, hereditary developed, life-threatening sickness of the blood categorized by obliteration of red blood cells by the accompaniment system, a part of the body's inherent immune system.


Symptoms
The definitive sign of PNH is red staining of the urine due to the attendance of hemosiderin and hemoglobin from the failure of red blood cells.

Diagnosis
Blood tests in PNH show vicissitudes reliable with intravascular hemolytic anemia: low hemoglobin, raised lactate dehydrogenase, raised bilirubin (a breakdown product of hemoglobin), and decreased levels of haptoglobin.

Treatment

Transfusion therapy may be needed, in adding to modifying important anemia, this overpowers the making of PNH cells by the bone marrow.








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