PAROXYSMAL NOCTURNAL HEMOGLOBINURIA: CASE REPORT WITH COMMENTS UPON THE URINARY IRON LOSS
Authors: S I RAPAPORT, E B REILLY, N R EADE, H O CARNE
Journal: Annals of Internal Medicine (1956)

Case Reports1 April 1956PAROXYSMAL NOCTURNAL HEMOGLOBINURIA: CASE REPORT WITH COMMENTS UPON THE URINARY IRON LOSSSAMUEL I. RAPAPORT, M.D., EMMETT B. REILLY, M.D., NORMAN R. EADE, M.D., HERBERT O. CARNE, M.S.SAMUEL I. RAPAPORT, M.D.Search for more papers by this author, EMMETT B. REILLY, M.D.Search for more papers by this author, NORMAN R. EADE, M.D.Search for more papers by this author, HERBERT O. CARNE, M.S.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-44-4-812 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptParoxysmal 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...Bibliography1. PillemerBlumLepowRossToddWardlow LLIHOAEWAC: The properdin system and immunity: I. Demonstration and isolation of a new serum protein, properdin, and its role in immune phenomena, Science 120: 279-285 (Aug.) 1954. CrossrefMedlineGoogle Scholar2. Ham TH: Studies on destruction of red blood cells; chronic hemolytic anemia with paroxysmal nocturnal hemoglobinuria: investigation of mechanism of hemolysis, with observations on 5 cases, Arch. Int. Med. 64: 1271-1305 (Dec.) 1939. CrossrefGoogle Scholar3. Crosby WH: Paroxysmal nocturnal hemoglobinuria. A specific test for the disease based on the ability of thrombin to activate the hemolytic factor, Blood 5: 843-846 (Sept.) 1950. CrossrefMedlineGoogle Scholar4. Crosby WH: Paroxysmal nocturnal hemoglobinuria. Relation of the clinical manifestations to underlying pathogenic mechanisms, Blood 8: 769-812 (Sept.) 1953. CrossrefMedlineGoogle Scholar5. QuickGeorgatsosHussey AJJGCV: The clotting activity of human erythrocytes: theoretical and clinical implications, Am. J. M. Sc. 228: 207-213 (Aug.) 1954. CrossrefMedlineGoogle Scholar6. KitzesElvehjemSchuette GCAHA: Determination of blood plasma iron, J. Biol. Chem. 155: 653-660 (Oct.) 1944. CrossrefGoogle Scholar7. Granick S: Iron metabolism and hemochromatosis, Bull. New York Acad. Med. 25: 403-428 (July) 1949. MedlineGoogle Scholar8. HickeyMalley MDLK: Chronic haemolytic anemia with hemoglobinuria, Quart. J. Med. 17: 1-8 (Jan.) 1948. MedlineGoogle Scholar9. Dacie JV: Transfusion of saline washed red cells in nocturnal hemoglobinuria (Marchiafava-Micheli disease), Clin. Sc. 7: 65-75 (July) 1948. MedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: Long Beach, California*Received for publication January 24, 1955.From the Department of Internal Medicine and the Laboratory Service, Veterans Administration Hospital, Long Beach, California. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byParoxysmal Nocturnal Hemoglobinuria "Obscured" by the Presence of Iron DeficiencyHERBERT E. KANN JR., M.D., CHARLES E. MENGEL, M.D., ROBERT L. WALL, M.D., F.A.C.P.Paroxysmal nocturnal haemoglobinuria 1 April 1956Volume 44, Issue 4Page: 812-818KeywordsAnemiaBlood plasmaExcretionHemoglobinHemolytic anemiaHospital medicineIngestionRed blood cellsResearch laboratoriesThrombosis Issue Published: 1 April 1956 PDF downloadLoading ...