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Marchiafava-micheli syndrome

Marchiafava-micheli syndrome --> paroxysmal nocturnal haemoglobinuria

An infrequent disorder with insidious onset (usually in the third or fourth decade) and chronic course, characterised by episodes of haemolytic anaemia, haemoglobinuria (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 cell membrane which makes the red cell unusually sensitive to lysis by complement.

Synonym: marchiafava-micheli anaemia, marchiafava-micheli syndrome.


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