A THROMBASTHENIC SYNDROME ASSOCIATED WITH HYPERHEPARINEMIA
Authors: W N BELL, I IMBER
Journal: Annals of Internal Medicine (1957)

Article1 March 1957A THROMBASTHENIC SYNDROME ASSOCIATED WITH HYPERHEPARINEMIAWARREN N. BELL, M.D., D.SC. (Med.), F.R.C.P.(C), IRVING IMBER, M.D., F.A.C.P.WARREN N. BELL, M.D., D.SC. (Med.), F.R.C.P.(C)Search for more papers by this author, IRVING IMBER, M.D., F.A.C.P.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-46-3-537 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptDefective platelet function has been postulated as the basic defect in pseudohemophilia.17This is a hereditary hemorrhagic diathesis affecting either sex and characterized by a prolonged bleeding time, normal platelet count and normal clotting time. An intermittent course and spontaneous improvement in later life have been noted. A somewhat similar clinical picture has also been designated as "thrombasthenia" by other authors. Our results in a group of 27 such patients, using more definitive coagulation tests, demonstrated variable platelet dysfunction. Accordingly, we prefer the term "thrombasthenia" for such a syndrome, since it appears more descriptive. All patients demonstrated hyperheparinemia during active...Bibliography1. Glanzmann E: Hereditare hemorrhagische Thrombasthenie, Ann. pædiat. 88: 113, 1918. Google Scholar2. Hirschboek JS: The effect of operation and illness on clot retraction—description of a new method, J. Lab. and Clin. Med. 33: 347, 1948. MedlineGoogle Scholar3. MooltenVroman SEL: The measurement of platelet adhesiveness by the glass wool filter, Am. J. Clin. Path. 19: 701, 1949. CrossrefMedlineGoogle Scholar4. AllenMoulderElghammerGrossmanMcKeenSandersonEgnerCrosbie JGPVRMBJCLMWJM: A protamine titration as an indication of a clotting defect in certain hemorrhagic states, J. Lab. and Clin. Med. 34: 473, 1949. MedlineGoogle Scholar5. LeRoyGalpernDolkart GVBRE: An indirect, quantitative method for the estimation of heparin activity in vitro, J. Lab. and Clin. Med. 35: 446, 1950. Google Scholar6. GibsonCarrGreenFowler RBTLSWM: Photometric assay of plasma heparin, Proc. Soc. Exper. Biol. and Med. 79: 577, 1952. CrossrefMedlineGoogle Scholar7. Stefanini M: Quantitative procedure for labile factor, Am. J. Clin. Path. 20: 233, 1950. CrossrefMedlineGoogle Scholar8. BaileyMcAlpin FRK: Familial purpura: 2 cases, Am. J. M. Sc. 190: 263, 1935. CrossrefGoogle Scholar9. BalesHartnett AW: Pseudohemophilia in a colored male, Am. Pract. and Digest Treat. 1: 570, 1950. MedlineGoogle Scholar10. Bell WN: A coagulation defect due to an anticoagulant possessing antithromboplastic and antithrombic properties, probably heparin, Blood 6: 1199, 1951. CrossrefMedlineGoogle Scholar11. BellStuart WNEG: Use of desoxycorticosterone acetate in treatment of "hyperheparinemia," Proc. Soc. Exper. Biol. and Med. 77: 550, 1951. CrossrefMedlineGoogle Scholar12. Macfarlane RG: The mechanism of hemostasis, Quart. J. Med. 10: 1, 1941. Google Scholar13. EstrenMedalDameshek SLSW: Pseudohemophilia, Blood 1: 504, 1946. CrossrefMedlineGoogle Scholar14. SavitskyWerman JPR: Platelet adhesiveness and clot retraction time, Am. J. Clin. Path. 22: 1175, 1952. CrossrefMedlineGoogle Scholar15. StefaniniCrosby MWH: The one-stage prothrombin consumption test, Blood 5: 964, 1950. CrossrefMedlineGoogle Scholar16. JurgensForsins RH: Studies on constitutional thrombopathy, Schweiz. med. Wchnschr. 81: 1248, 1951. MedlineGoogle Scholar17. Von Willebrand EA: Über hereditäre Pseudohemophilie, Acta med. Scandinav. 76: 521, 1931. CrossrefGoogle Scholar18. Fonio A: Über das funktionelle Verhalten der Thrombozyten beim Gerinnungsvorgang, Die Medizinische 1: 1, 1952. Google Scholar19. CastexPavlovsky MRA: Pseudohemophilia due to an excess of anticoagulant substances of heparin type, Sang 18: 1, 1947. MedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: *Presented at the Thirty-seventh Annual Session of The American College of Physicians, Los Angeles, California, April 17, 1956.Part of this work was carried out during the tenure of a Damon Runyon Fellowship for Cancer Research at the Hospital of the University of Pennsylvania, Philadelphia, and part during tenure of a Storey Fellowship for Leukemia Research at the University of Cambridge, England, by one of us (W. N. B.). The work was aided in part by a grant from Eli Lilly and Company, of Indianapolis.Requests for reprints should be addressed to Warren N. Bell, M.D., Division of Hematology, Department of Medicine, University Hospital, Jackson, Mississippi. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byBIBLIOGRAPHYAngiohämophilie 1 March 1957Volume 46, Issue 3Page: 537-545KeywordsFellowshipsHemorrhagePlatelets ePublished: 1 December 2008 Issue Published: 1 March 1957 PDF downloadLoading ...