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paroxysmal nocturnal hemoglobinuria

 

Definition: an infrequent disorder with insidious onset (usually in the third or fourth decade) and chronic course, characterized by episodes of hemolytic anemia, hemoglobinuria (chiefly at night), pallor, icterus or bronzing of the skin, a moderate degree of splenomegaly, and sometimes hepatomegaly; red blood cells are usually macrocytic and vary considerably in size, but there is no evidence of spherocytosis, erythrophagocytosis, or abnormal leukocytes. The disorder is a result of an abnormality of the red blood cell membrane, which makes the red blood cell unusually sensitive to lysis by complement.

Synonym(s): Marchiafava-Micheli anemia, Marchiafava-Micheli syndrome

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© Copyright 2017 Wolters Kluwer. All Rights Reserved. Review Date: Sep 19, 2016.
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