Marchiafava-Micheli syndrome


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hemoglobinuria

 [he″mo-glo″bĭ-nu´re-ah]
the presence of free hemoglobin in the urine. adj., adj hemoglobinu´ric.
march hemoglobinuria hemolysis caused by repeated uncushioned shocks or trauma to some body part, such as in some soldiers on long marches, in marathon runners, and in karate practitioners.
paroxysmal cold hemoglobinuria an autoimmune or postviral disease in which there is a biphasic IgG antibody directed against the P blood group antigen. It is marked by episodes of hemoglobinemia and hemoglobinuria after exposure to cold and is detected by the Donath-Landsteiner test. The condition is treated with prednisone and cyclophosphamide and by protection from exposure to cold.
paroxysmal nocturnal hemoglobinuria (PNH) an acquired blood cell abnormality with proliferation of abnormal red blood cells (PNH cells) that are readily hemolyzed by complement, and episodes of severe hemolysis and thrombosis, particularly of the hepatic veins. It is detected by the ham test. Treatment is with androgens or prednisone and, during thrombotic episodes, with heparin.

par·ox·ys·mal noc·tur·nal he·mo·glo·bi·nu·ri·a

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.

Marchiafava-Micheli syndrome

(măr″kē-ă-fă′vă-mē-kā′lē)
[Ettore Marchiafava, It. pathologist, 1847–1935; F. Micheli, It. clinician, 1872–1937]
A rare hemolytic anemia associated with paroxysmal nocturnal hemoglobinuria.

Marchiafava,

Ettore, Italian pathologist, 1847-1935.
Marchiafava syndrome - Synonym(s): Marchiafava-Bignami syndrome
Marchiafava-Bignami disease - a disorder consisting of demyelination of the corpus callosum and cortical laminar necrosis involving the frontal and temporal lobes. Synonym(s): Marchiafava syndrome
Marchiafava-Micheli anemia - Synonym(s): Marchiafava-Micheli syndrome
Marchiafava-Micheli syndrome - an infrequent disorder with insidious onset and chronic course, characterized by episodes of hemolytic anemia, hemoglobinuria, pallor, icterus or bronzing of the skin, a moderate degree of splenomegaly, and sometimes hepatomegaly. Synonym(s): Marchiafava-Micheli anemia; paroxysmal nocturnal hemoglobinuria

Micheli,

Ferdinando, Italian physician, 1872-1936.
Marchiafava-Micheli anemia - Synonym(s): Marchiafava-Micheli syndrome
Marchiafava-Micheli syndrome - see under Marchiafava