paroxysmal nocturnal hemoglobinuria
- paroxysmal nocturnal hemoglobinuria n a form of hemolytic anemia that is characterized by an abnormally strong response to the action of complement, by acute episodes of hemolysis esp. at night with hemoglobinuria noted upon urination after awakening, venous occlusion, and often leukopenia and thrombocytopenia abbr. PNH
* * *(PNH) a chronic acquired blood cell dysplasia with proliferation of a clone of stem cells producing erythrocytes, platelets, and granulocytes that are abnormally susceptible to lysis by complement; it is marked by episodes of intravascular hemolysis, causing hemolytic anemia, particularly following infections, and by venous thromboses, especially of the hepatic veins. Diagnosis is based on the acidified serum test (Ham test) or the sucrose lysis test. Called also Marchiafava-Micheli syndrome.
Medical dictionary. 2011.
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