PAROXYSMAL NOCTURNAL HEMOGLOBINURIA: REPORT OF A CASE COMPLICATED BY AN AREGENERATIVE (APLASTIC) CRISIS
Authors: William H. Crosby
Journal: Annals of Internal Medicine (1953)

Case Reports1 November 1953PAROXYSMAL NOCTURNAL HEMOGLOBINURIA: REPORT OF A CASE COMPLICATED BY AN AREGENERATIVE (APLASTIC) CRISISWILLIAM H. CROSBYWILLIAM H. CROSBYSearch for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-39-5-1107 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptParoxysmal nocturnal hemoglobinuria (PNH) is an uncommon disease, but not so rare as has been supposed. At least 164 case reports have appeared in print, 28 of them since January, 1950.†PNH is manifested by a chronic hemolytic disease that becomes more active when the patient sleeps. During a hemolytic crisis of moderate intensity the destruction of red cells during sleep may result in "nocturnal" hemoglobinuria that disappears shortly after the patient has been awake for several hours. During severe crises hemoglobinuria may persist day and night. In the absence of crises the patient with PNH may live for years...Bibliography1. BartaGörög ID: Splenomegalia hämolytica mit paroxysmaler Hämoglobinurie, Virchows Arch. f. path. Anat. 273: 266, 1929. CrossrefGoogle Scholar2. Bremy P: Die Gewebsmastzellen im menschlichen Knochenmark, 1950, Georg Thieme Verlag, Stuttgart. Google Scholar3. ClappWilliamsMendel MPMJJL: Observations on the hemolytic mechanism of paroxysmal nocturnal hemoglobinuria. Demonstration of the activity of at least two serum factors in PNH hemolysis, Blood 7: 1117, 1952. CrossrefMedlineGoogle Scholar4. Crosby WH: Paroxysmal nocturnal hemoglobinuria. A classic description by Paul Strübing in 1882 and a bibliography of the disease, Blood 6: 270, 1951. CrossrefMedlineGoogle Scholar5. Crosby WH: Paroxysmal nocturnal hemoglobinuria. Plasma factors of the hemolytic system, Blood 8: 444, 1953. CrossrefMedlineGoogle Scholar6. Crosby WH: Paroxysmal nocturnal hemoglobinuria. Relation of the clinical manifestations to underlying pathogenic mechanisms, Blood 8: 769 (Sept.) 1953. CrossrefMedlineGoogle Scholar7. CrosbyDameshek WHW: Paroxysmal nocturnal hemoglobinuria. The mechanism of hemolysis and its relation to the coagulation mechanism, Blood 5: 822, 1950. CrossrefMedlineGoogle Scholar8. CrosbyStefanini WHM: Pathogenesis of the plasma transfusion reaction with especial reference to the blood coagulation system, J. Lab. and Clin. Med. 40: 374, 1952. Google Scholar9. DacieIsraëlsWilkinson JVMCJF: Paroxysmal nocturnal haemoglobinuria of the Marchiafava type, Lancet 1: 479, 1938. CrossrefGoogle Scholar10. Dameshek W: Paroxysmal nocturnal hemoglobinuria (Marchiafava-Micheli syndrome), Bull. New England M. Center 4: 224, 1942. Google Scholar11. DameshekNeber WJ: Transfusion reactions to a plasma constituent of whole blood. Their pathogenesis and treatment by washed red blood cell transfusions, Blood 5: 129, 1950. CrossrefMedlineGoogle Scholar12. EllenhornFeigenbaumPlumhofMettier MJLZCSR: Paroxysmal nocturnal hemoglobinuria with chronic hemolytic anemia. Report of a case with postmortem observations, Arch. Int. Med. 87: 868, 1951. CrossrefGoogle Scholar13. Ham TH: Studies on destruction of red blood cells. I. Chronic hemolytic anemia with paroxysmal nocturnal hemoglobinuria: an investigation of the mechanism of hemolysis, with observations on five cases, Arch. Int. Med. 64: 1271, 1939. CrossrefGoogle Scholar14. KalantCyr NDP: Treatment of paroxysmal nocturnal hemoglobinuria with ACTH, Blood 7: 607, 1952. CrossrefMedlineGoogle Scholar15. LarraínDel SolarMaccioniPérez CAAA: Anemia hemolítica crónica con hemoglobinuria nocturna paroxística (Enfermedad de Marchiafava-Micheli), Rev. méd. de Chile 78: 735, 1950. MedlineGoogle Scholar16. Maier C: Hämolyse und hämolytische Krankheiten, 1950, Hans Huber, Bern, p. 194. Google Scholar17. Merliss RR: Paroxysmal nocturnal hemoglobinuria. A clinicopathological correlation, New England J. Med. 246: 642, 1952. CrossrefMedlineGoogle Scholar18. Owren PA: Congenital hemolytic jaundice. The pathogenesis of the "hemolytic crisis," Blood 3: 231, 1948. CrossrefMedlineGoogle Scholar19. PantonMaitland-JonesRiddoch PNAGG: The relationship of some hemolytic disorders to pernicious anaemia, Lancet 1: 529, 1924. CrossrefGoogle Scholar20. SingerMotulskyWile KAGSA: Aplastic crisis in sickle cell anemia. A study of its mechanism and its relationship to other types of hemolytic crisis, J. Lab. and Clin. Med. 35: 721, 1950. MedlineGoogle Scholar21. Baines A: Paroxysmal haematinuria, Canadian Practitioner 11: 33, 1886. Google Scholar22. BangGeorg HOJ: Paroxystic haemoglobinuri hos en patient med ovalocytose, Nord. med. 35: 1827, 1947. Google Scholar23. CarlingCarlillPulvertaft ERHRJ: Splenectomy in Gaucher's disease with haemoglobinuria, Proc. Roy. Soc. Med. 26: 361, 1932. CrossrefGoogle Scholar24. Chapin WB: A case of haematuria in the male, resembling in its periodical recurrence the menstrual epoch, New York Med. J. 49: 378, 1889. Google Scholar25. Charpentier AE: A case of paroxysmal haematinuria, with functional albuminuria, Lancet 2: 161, 1888. CrossrefGoogle Scholar26. Emerson CP: Unilateral paroxysmal haemoglobinuria, Johns Hopkins Hosp. Bull. 17: 238, 1906. Google Scholar27. Goelet AH: A case of paroxysmal haematinuria, with unusual irregularity of the paroxysms, Am. J. M. Sc. 77: 285, 1879. CrossrefGoogle Scholar28. Haden RL: Hemolytic anemia, J. Lab. and Clin. Med. 26: 65, 1940. Google Scholar29. Herringham WP: Paroxysmal haematuria, St. Bartholomew's Hosp. Report, London 22: 133, 1886. Google Scholar30. Johnson SR: Indications for splenectomy illustrated by means of spleen extirpations carried out at the surgical clinic of the Karolinska Sjukhuset from 1940-1947, Acta chir. Scandinav. 99: 1, 1949. MedlineGoogle Scholar31. Morrill FG: A case of haemoglobinuria, Boston M. and S. J. 106: 108, 1882. CrossrefGoogle Scholar32. SauvageVincent : Anémie pernicieuse de la grossesse, Bull. Soc. gynec. et obst. 2: 156, 1913. Google Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: *Received for publication July 10, 1953.From the Department of Hematology, Army Medical Service Graduate School, Walter Reed Army Medical Center, Washington, D. C., and The Medical Service, Brooke Army Hospital, Fort Sam Houston, Texas.†It was stated in a recent review of the literature that 123 cases had been reported as of January, 1950.4 One of the 123 (Malmejac et al.) did not deal with PNH. Meanwhile, 12 additional older case reports have been identified.21-32 One of these, published by Goelet in 1879, probably represents the first report of PNH to be published in the United States. The diagnosis was not made because PNH had not yet been established as an entity. In identifying this and other old case reports with PNH, the total medical history of the patient was consistent with the diagnosis and, in addition, there was described the appearance of pigment in the urine following sleep, or characteristic tests in vitro were found to be positive. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byChronic parvovirus infection and G6PD deficiency masquerading as Diamond-Blackfan anemiaMRI findings in renal cortical hemosiderosisHepatic vein thrombosis in paroxysmal nocturnal hemoglobinuriaAPLASTIC CRISES IN HAEMOLYTIC ANAEMIAS: THE ROLE OF A PARVOVIRUS-LIKE AGENTParoxysmal Nocturnal HaemoglobinuriaParoxysmal Nocturnal Haemoglobinuria and Refractory Marrow Failure Treated by Marrow TransplantationParoxysmale n�chtliche H�moglobinurie mit terminaler Niereninsuffizienz und Erl�schen der spezifischen Krankheitssymptome (Langzeitverlauf und pathologisch-anatomische Befunde)Teilungsstörungen der Erythropoese: Perniciosa-Syndrom und Erythroblastopenie-SyndromIntestinal Infarction in Paroxysmal Nocturnal HemoglobinuriaMegaloblastic Crisis in Paroxysmal Nocturnal HemoglobinuriaParoxysmal Nocturnal Hemoglobinuria Following Drug-induced Aplastic AnemiaJ. M. QUAGLIANA, M.D., G. E. CARTWRIGHT, M.D., M. M. WINTROBE, M.D., PH.D., F.A.C.P.Paroxysmal nocturnal hemoglobinuria presenting as aplastic anemia in a childPancytopenia and Bone Marrow Hypoplasia in a Case of Paroxysmal Nocturnal HemoglobinuriaParoxysmal Nocturnal Haemoglobinuria: Variation in Clinical Severity and Association with Bone-Marrow HypoplasiaTHE APLASTIC CRISIS IN SICKLE-CELL ANÆMIAAcquired hemolytic anemia and transient erythroid hypoplasia of bone marrowBone-Marrow Failure Due to Relative Nutritional Deficiency in Cooley's Hemolytic AnemiaZur Therapie der paroxysmalen n�chtlichen H�moglobinurieProperdin und H�molyse 1 November 1953Volume 39, Issue 5Page: 1107-1117KeywordsCellsGraduate medical educationHematology and oncologyHemoglobinMedical servicesSleepUrine ePublished: 1 December 2008 Issue Published: 1 November 1953 PDF downloadLoading ...