SAMUEL I. RAPAPORT, M.D.; EMMETT B. REILLY, M.D.; NORMAN R. EADE, M.D.; HERBERT O. CARNE, M.S.
Paroxysmal nocturnal hemoglobinuria (Marchiafava-Micheli syndrome) is a rare chronic hemolytic anemia characterized by intravascular hemolysis and the urinary excretion of hemoglobin and hemosiderin. An acquired defect of the red blood cell stroma causes the patient's erythrocytes to be destroyed by a normal plasma factor or factors, possibly the newly described euglobulin, properdin.1 An associated leukopenia and thrombocytopenia suggest that these elements share the protoplasmic defect. Despite the thrombocytopenia, spontaneous thromboses are common and a major cause of death.
This case report illustrates many of the unique features of paroxysmal nocturnal hemoglobinuria. It calls particular attention to the possibility of the
RAPAPORT SI, REILLY EB, EADE NR, et al. PAROXYSMAL NOCTURNAL HEMOGLOBINURIA: CASE REPORT WITH COMMENTS UPON THE URINARY IRON LOSS*. Ann Intern Med. 1956;44:812–818. doi: https://doi.org/10.7326/0003-4819-44-4-812
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Published: Ann Intern Med. 1956;44(4):812-818.
Hematology/Oncology, Red Cell Disorders.
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