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Brief Reports1 November 1977Treatment of Acquired C1-Inhibitor Deficiency with DanazolGEORGES HAUPTMANN, M.D., SIMONE MAYER, M.D., JEAN-MARIE LANG, M.D., FRANCIS OBERLING, M.D., GEORGES MAYER, M.D.GEORGES HAUPTMANN, M.D.Search for more papers by this author, SIMONE MAYER, M.D.Search for more papers by this author, JEAN-MARIE LANG, M.D.Search for more papers by this author, FRANCIS OBERLING, M.D.Search for more papers by this author, GEORGES MAYER, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-87-5-577 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptHereditary angioedema is characterized by a marked deficiency of the inhibitor of activated first component of complement (C1 inhibitor). Some cases of acquired C1-inhibitor deficiencies and angioedema have been reported in association with lymphoproliferative diseases (1-4). An acquired C1-inhibitor deficiency ("Caldwell's syndrome") can be distinguished from hereditary angioedema by the absence of C1-inhibitor deficiency in other family members, by the profound reduction of C1 level as well as reduction of C4 and C2 levels, and by the presence of, in most cases, serum immunoglobulin abnormalities (high IgG of IgM levels, presence of low molecular weight IgM) and high C1-depleting activity...References1. CALDWELLRUDDYSCHURAUSTEN JSPK: Acquired C1 inhibitor deficiency in lymphosarcoma. Clin Immunol Immunopathol 1:39-52, 1972 CrossrefGoogle Scholar2. ROSENFELDSTAPLESLEDDY SPJ: Angioedema and hypocomplementemia: unusual features of lymphoma. J Allergy Clin Immunol 55:104, 1975 Google Scholar3. HAUPTMANNLANGNORTHOBERLINGMAYERLACHMANN GJMFGP: Acquired C1-inhibitor deficiencies in lymphoproliferative diseases with serum immunoglobulin abnormalities. A study of three cases. Blut 32:195-206, 1976 CrossrefMedlineGoogle Scholar4. SCHREIBERZWEIMANATKINSGOLDWEINPIETRAATKINSONABDOU ABPFGBN: Acquired angioedema with lymphoproliferative disorder: association of C1 inhibitor deficiency with cellular abnormality. Blood 48:567-580, 1976 CrossrefMedlineGoogle Scholar5. GELFANDSHERINSALLINGFRANK JRDM: Treatment of hereditary angioedema with Danazol. N Engl J Med 295: 1444-1448, 1976 CrossrefMedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAuthors: GEORGES HAUPTMANN, M.D.; SIMONE MAYER, M.D.; JEAN-MARIE LANG, M.D.; FRANCIS OBERLING, M.D.; GEORGES MAYER, M.D.Affiliations: PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byCase report presenting the diagnostic challenges in a patient with recurrent acquired angioedema, antiphospholipid antibodies and undetectable C2 levelsAcquired C1 Inhibitor DeficiencyAcquired Deficiency of the Inhibitor of the First Complement Component: Presentation, Diagnosis, Course, and Conventional ManagementAutoantibodies and Lymphoproliferative Diseases in Acquired C1-Inhibitor DeficienciesLymphoproliferative disease causing angioedema - an uncommon associationAngioedema associated with C1 inhibitor deficiencyPathologyLes angio-œdèmes par déficit en C1 inhibiteurAcquired angioedemaAcquired angioedema as the presenting feature of lymphoproliferative disorders of mature B-lymphocytesAutoimmune C1 inhibitor deficiency: Report of eight patientsComplement activation during bypass in acquired C1 esterase inhibitor deficiencyLymphocytic lymphoma and acquired C1 esterase inhibitor deficiencyAcquired C 1 esterase inhibitor deficiencyEffect of Danazol on Solubilization of Immune Deposits in Patients With IgA NephropathyDanazolAMOS E. MADANES, M.D., MARTIN FARBER, M.D.Effect of danazol on dysfunctional C 1 inhibitor in a patient with angioedemaUrticaria/AngioedemaAddendumLOW 1,25-DIHYDROXYVITAMIN D IN HEPARIN-INDUCED OSTEOPENIAREVERSAL OF LUPUS-ERYTHEMATOSUS-LIKE DISEASE WITH DANAZOLAcquired Angioedema and Hypocomplementemia in a Patient with MyelofibrosisThe Regulation of Complement Activity by Pharmacologic AgentsURTICARIA/ANGIOEDEMA A CONSIDERATION OF PATHOCENESIS AND CLINICAL MANIFESTATIONSBilateral carpal tunnel syndrome and arthritis associated with danazol administrationDanazol® and C1 Esterase Inhibitor DeficiencySTEVEN H. COHEN, M.D., SUSAN M. KOETHE, PH.D. 1 November 1977Volume 87, Issue 5Page: 577-578KeywordsHereditary angioedema ePublished: 1 December 2008 Issue Published: 1 November 1977 PDF downloadLoading ...

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