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Case Studies1 June 1967Thrombotic Thrombocytopenic PurpuraSerial Clotting Studies, Relation to the Generalized Shwartzman Reaction, and Remission After Adrenal Steroid and Dextran TherapyROBERT G. LERNER, M.D., SAMUEL I. RAPAPORT, M.D., F.A.C.P., JAMES MELTZER, M.D.ROBERT G. LERNER, M.D.Search for more papers by this author, SAMUEL I. RAPAPORT, M.D., F.A.C.P.Search for more papers by this author, JAMES MELTZER, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-66-6-1180 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptThrombotic thrombocytopenic purpura (TTP) was first described by Moschcowitz (1) in 1925. It is an acute, fluctuating illness whose major clinical manifestations are thrombocytopenia, hemolytic anemia with characteristically fragmented red blood cells, changing neurologic findings, fever, jaundice with both direct and indirect bilirubinemia, and renal abnormalities. When a patient exhibits all six of these findings, one may make the diagnosis clinically—provided, however, that other disorders that may mimic TTP (for example, systemic lupus erythematosus or metastatic malignancy with disseminated intravascular coagulation) have been searched for carefully and eliminated. The pathologic diagnosis requires more than the demonstration of hyaline thrombi in...References1. MOSCHCOWITZ E: Acute febrile pleiochromic anemia with hyaline thrombosis of the terminal arterioles and capillaries: an undescribed disease. Arch. Intern. Med. (Chicago) 36: 89, 1925. CrossrefGoogle Scholar2. MCKAY DG: Disseminated Intravascular Coagulation: An Intermediary Mechanism of Disease. Hoeber Medical Division, Harper & Row, Publishers, New York, 1965. Google Scholar3. LUKESRATHSTEUSSYMAILLIARD RJCECNJ: Thrombotic thrombocytopenic purpura—clinical and pathological findings in 49 cases (abstract). Blood 17: 366, 1961. Google Scholar4. GORE I: Disseminated arteriolar and capillary platelet thrombosis. A morphologic study of its histogenesis. Amer. J. Path. 26: 155, 1950. MedlineGoogle Scholar5. ORBISON JL: Morphology of thrombotic thrombocytopenic purpura with demonstration of aneurysms. Amer. J. Path. 28: 129, 1952. MedlineGoogle Scholar6. TAUBRODRIGUEZ-ERDMANNDAMESHEK RNFW: Intravascular coagulation, the Shwartzman reaction and the pathogenesis of T. T. P. Blood 24: 775, 1964. CrossrefMedlineGoogle Scholar7. HJORTRAPAPORT PFSI: The Shwartzman reaction: pathogenetic mechanisms and clinical manifestations. Ann. Rev. Med. 16: 135, 1965. CrossrefMedlineGoogle Scholar8. WAREGUESTSEEGERS AGMMWH: Fibrinogen: with special reference to its preparation and certain properties of the product. Arch. Biochem. 13: 231, 1947. MedlineGoogle Scholar9. HJORTRAPAPORTOWREN PSIPA: A simple, specific one-stage prothrombin assay using Russell's viper venom in cephalin suspension. J. Lab. Clin. Med. 46: 89, 1955. MedlineGoogle Scholar10. RAPAPORTAMESMIKKELSEN SISBS: The levels of antihemophilic globulin and proaccelerin in fresh and bank blood. Amer. J. Clin. Path. 31: 297, 1959. CrossrefMedlineGoogle Scholar11. RAPAPORTAASOWREN SIKPA: The effect of glass upon the activity of the various plasma clotting factors. J. Clin. Invest. 34: 9, 1955. CrossrefMedlineGoogle Scholar12. SCHIFFMANRAPAPORTPATCH SSIMJ: Starch block electrophoresis of plasma and serum clotting factors. Separation of activated PTC (PTC′). Blood 25: 724, 1965. CrossrefMedlineGoogle Scholar13. RAPAPORTAMES SISB: Relation between levels of plasma thromboplastin component (PTC) and prothrombin times by the P & P and Quick methods in patients receiving warfarin. New Eng. J. Med. 267: 125, 1962. CrossrefMedlineGoogle Scholar14. HOUGIE C: A simple assay method for factor X (Stuart-Prower factor). Proc. Soc. Exp. Biol. Med. 109: 754, 1962. CrossrefMedlineGoogle Scholar15. RAPAPORTSCHIFFMANPATCHWARE SISMJAG: A simple, specific one-stage assay for plasma thromboplastin antecedent activity. J. Lab. Clin. Med. 57: 771, 1961. MedlineGoogle Scholar16. SCHIFFMANRAPAPORTWAREMEHL SSIAGJW: Separation of plasma thromboplastin antecedent (PTA) and Hageman factor (HF) from human plasma. Proc. Soc. Exp. Biol. Med. 105: 453, 1960. CrossrefMedlineGoogle Scholar17. BRAYTONZUCKER RGMB: Bleeding syndrome in thrombin-injected rats deficient in fibrinogen and antihemophilic factor. Proc. Soc. Exp. Biol. Med. 96: 418, 1957. CrossrefMedlineGoogle Scholar18. KOWALSKIBUDZYŃSKIKOPEĆLATALLOLIPINSKIWEGRZYNOWICZ EAZMZSBZ: Circulating fibrinogen degradation products (FDP) in dog blood after intravenous thrombin infusion. Thromb. Diath. Haemorrh. 13: 12, 1965. MedlineGoogle Scholar19. RODRIGUEZ-ERDMANN F: Bleeding due to increased intravascular blood coagulation. Hemorrhagic syndromes caused by consumption of blood-clotting factors (consumption coagulopathies). New Eng. J. Med. 273: 1370, 1965. CrossrefMedlineGoogle Scholar20. IATRIDISFERGUSON PGJH: Plasminoplastin generation test of normal, HF-, PTA-, X-, PTC- and AHF- platelet-poor plasmas: evidence that only HF- plasma has an abnormal fibrinolytic activity. Nature (London) 207: 1404, 1965. CrossrefMedlineGoogle Scholar21. IATRIDISFERGUSON SGJH: Effect of surface and Hageman factor on the endogenous or spontaneous activation of the fibrinolytic system. Thromb. Diath. Haemorrh. 6: 411, 1961. MedlineGoogle Scholar22. JAMESMONTO TNRW: Pathology of the cardiac conduction system in thrombotic thrombocytopenic purpura. Ann. Intern. Med. 65: 37, 1966. LinkGoogle Scholar23. BRAINDACIEHOURIHANE MCJVDO: Microangiopathic haemolytic anaemia: the possible role of vascular lesions in pathogenesis. Brit. J. Haemat. 8: 358, 1962. CrossrefMedlineGoogle Scholar24. BRITTINGHAMCHAPLIN TEH: Attempted passive transfer of thrombotic thrombocytopenic purpura. Blood 12: 480, 1957. CrossrefMedlineGoogle Scholar25. ADELSONSTEFANINI EM: Studies on platelets. VI. Demonstration and characterization of a heterologous (Forssman) platelet agglutinin. Blood 7: 700, 1952. CrossrefMedlineGoogle Scholar26. SCHAEFFERCOLLINGS WEH: Thrombotic thrombocytopenic purpura. Report of a case and review of the literature. Guthrie Clin. Bull. 29: 181, 1960. Google Scholar27. Clinicopathological Conference: Ohio Med. J. 61: 148, 1965. MedlineGoogle Scholar28. PESSIN SB: Clinicopathologic conference sponsored by the Section on Pathology, State Medical Society of Wisconsin. Wisconsin Med. J. 63: 569, 1964. MedlineGoogle Scholar29. PARSONSTWEEDDALELARSEN DWDNFI: Thrombotic thrombocytopenic purpura: report of a case. J. Kentucky Med. Ass. 60: 856, 1962. MedlineGoogle Scholar30. Clinicopathologic Conference: Thrombotic thrombocytopenic purpura. J. Tenn. Med. Ass. 56: 290, 1963. Google Scholar31. BORNSTEINBOSSCASPERBEHAR BJHJM: Thrombotic thrombocytopenic purpura. Report of a case presenting as a chronic neurological disorder and characterized by unusual histological findings. J. Clin. Path. 13: 124, 1960. CrossrefMedlineGoogle Scholar32. FINESTONELAWRENCE AJJW: Thrombotic thrombocytopenic purpura. Intestinal hemorrhage as a cardinal manifestation. Arch. Intern. Med. (Chicago) 107: 750, 1961. CrossrefMedlineGoogle Scholar33. BROWNTAYLOR JRF: Thrombotic thrombocytopenic purpura. Canad. Med. Ass. J. 89: 613, 1963. MedlineGoogle Scholar34. KENNEDY A: Thrombotic thrombocytopenic purpura. Canad. Med. Ass. J. 91: 450, 1964. MedlineGoogle Scholar35. ANTES EH: Thrombotic thrombocytopenic purpura: a review of the literature with report of a case. Ann. Intern. Med. 48: 512, 1958. LinkGoogle Scholar36. KNOXYOUNG FHJM: Thrombotic thrombocytopenic purpura. J. Tenn. Med. Ass. 52: 455, 1959. Google Scholar37. MCCORMACKO'BRIENOLIVER PDJRA: Two cases of thrombohaemolytic thrombocytopenic purpura associated with changes in red cell morphology. J. Clin. Path. 16: 436, 1963. CrossrefMedlineGoogle Scholar38. GREENROSENTHAL MAS: Generalized blood platelet thrombosis. Report of three cases with necropsy findings. J. Mount Sinai Hosp. N. Y. 16: 110, 1949. MedlineGoogle Scholar39. HILLLOEB JME: Massive hormonal therapy and splenectomy in acute thrombotic thrombocytopenic purpura; report of three cases and one eight-year cure. JAMA 173: 778, 1960. CrossrefGoogle Scholar40. BARNHARTMCCUTCHEONRIDDLEOHORODNIK MISAJMJM: Thrombotic thrombocytopenic purpura as a model of accelerated protein synthesis. Thromb. Diath. Haemorrh. 12: 211, 1964. MedlineGoogle Scholar41. DICKENMANBROWNHIRATZKAVETNE RCHJTG: Thrombotic thrombocytopenic purpura with associated afibrinogenemia. J. Mich. Med. Soc. 54: 1421, 1955. MedlineGoogle Scholar42. MERSKEYJOHNSONPERTWOHL CAJJHH: Pathogenesis of fibrinolysis in defibrination syndrome: effect of heparin administration. Blood 24: 701, 1964. CrossrefMedlineGoogle Scholar43. BUKOWSKIKOBLENZER MJPJ: Thrombotic thrombocytopenic purpura. Report of a case with the unusual features of hypofibrinogenemia and leukopenia. J. Pediat. 60: 84, 1962. CrossrefMedlineGoogle Scholar44. REISFIELD DR: Thrombotic thrombocytopenic purpura and pregnancy. Obstet. Gynec. Survey 14: 303, 1959. CrossrefMedlineGoogle Scholar45. AMOROSIULTMANN ELJE: Thrombotic thrombocytopenic purpura: report of 16 cases and review of the literature. Medicine (Balt.) 45: 139, 1966. CrossrefGoogle Scholar46. WEERDTVREEKEN CMJ: Influence of cryoprofibrin on the antiglobulin consumption test with platelets. Vox Sang. 10: 536, 1965. CrossrefMedlineGoogle Scholar47. ADELSONHEITZMANFENNESSEY EEJJF: Thrombohemolytic thrombocytopenic purpura. Arch. Intern. Med. (Chicago) 94: 42, 1954. CrossrefGoogle Scholar48. CAHALANEHORN SFRC: Thrombotic thrombocytopenic purpura of long duration. Amer. J. Med. 27: 333, 1959. CrossrefMedlineGoogle Scholar49. ALLANBYHUNTSMANSACKER KDRGLS: Thrombotic microangiopathy. Recovery of a case after heparin and magnesium therapy. Lancet 1: 237, 1966. CrossrefMedlineGoogle Scholar50. BERNSTOCKHIRSON LC: Thrombotic thrombocytopenic purpura. Remission on treatment with heparin. Lancet 1: 28, 1960. CrossrefMedlineGoogle Scholar51. BERNSTOCKHIRSON LC: Heparin in thrombotic microangiopathy. Lancet 2: 281, 1966. MedlineGoogle Scholar52. RODRIGUEZ-ERDMANNRUBINDAMESHEK FADW: Heparin in thrombotic microangiopathy. Lancet 1: 1033, 1966. CrossrefGoogle Scholar53. CARMICHAELMEDLEY DSDR: Heparin in thrombotic microangiopathy. Ibid., p. 1421. Google Scholar54. CRAIGGITLIN JMD: The nature of the hyaline thrombi in thrombotic thrombocytopenic purpura. Amer. J. Path. 33: 251, 1957. MedlineGoogle Scholar55. BURKEHARTMANN HARC: Thrombotic thrombocytopenic purpura. Arch. Intern. Med. (Chicago) 103: 105, 1959. CrossrefGoogle Scholar56. SHAPIRODOKTORCHURG HDDJ: Thrombotic thrombocytopenic purpura (Moschcowitz's disease): report of a case with remission after splenectomy and steroid therapy. Ann. Intern. Med. 47: 582, 1957. LinkGoogle Scholar57. THOMASGOOD LRA: The effect of cortisone on the Shwartzman reaction. The production of lesions resembling the dermal and generalized Shwartzman reactions by a single injection of bacterial toxin in cortisone-treated rabbits. J. Exp. Med. 95: 409, 1952. CrossrefMedlineGoogle Scholar58. BLOOMHARMERBRYANTBREWER WLDSMFSS: Coating of vascular surfaces and cells. A new concept in prevention of intravascular thrombosis. Proc. Soc. Exp. Biol. Med. 115: 384, 1964. CrossrefMedlineGoogle Scholar59. ROTHMANADELSONSCHWEBELLANGDELL SEARD: Adsorption of carbon-14 dextran to human blood platelets and red blood cells, in vitro. Vox Sang. 2: 104, 1957. MedlineGoogle Scholar60. LANGDELLADELSONFURTHCROSBY RDEFWWH: Dextran and prolonged bleeding time. JAMA 166: 346, 1958. CrossrefMedlineGoogle Scholar61. BRYANTBLOOMBREWER MFWLSS: Experimental study of the antithrombotic properties of dextrans of low molecular weight. Amer. Surg. 29: 256, 1963. MedlineGoogle Scholar62. PATTERSONPIERCEAMERSONWATKINS JHRBJRWL: Dextran therapy of purpura fulminans. New Eng. J. Med. 273: 734, 1965. CrossrefMedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAuthors: ROBERT G. LERNER, M.D.; SAMUEL I. RAPAPORT, M.D., F.A.C.P.; JAMES MELTZER, M.D.Affiliations: Los Angeles, CaliforniaFrom the Department of Medicine, the University of Southern California School of Medicine and the Los Angeles County General Hospital, Los Angeles, Calif.This study was supported by grants TI AM 05192-08 and HE 06128-06, the U. S. Public Health Service, Washington, D. C.At the time of this study Dr. Lerner was a trainee in the Graduate Training Program in Hematology, University of Southern California School of Medicine, Los Angeles, Calif.Requests for reprints should be addressed to Robert G. Lerner, M.D., University of Southern California School of Medicine, 2025 Zonal Ave., Los Angeles, Calif. 90033. 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THE GENERALIZED SHWARTZMAN REACTIONCase 29-1969Clinical pathologic conferenceDefibrination syndrome in a patient with chronic thrombocytopenic purpuraRecognition and treatment of intravascular coagulationTreatment of Patients with Microangiopathic Haemolytic Anaemia with HeparinMicroangiopathic Hemolytic Anemia Caused by Purpura FulminansJOHN H. HOLLINGSWORTH, M.D., DANIEL N. MOHLER, M.D., F.A.C.P. 1 June 1967Volume 66, Issue 6Page: 1180-1190KeywordsFeversGraduate medical educationHematology and oncologyHemolytic anemiaHospital medicineLupus erythematosusMedical servicesPartial thromboplastin timeRed blood cellsThrombotic thrombocytopenic purpura ePublished: 1 December 2008 Issue Published: 1 June 1967 PDF downloadLoading ...
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