GEORGES HAUPTMANN, M.D.; SIMONE MAYER, M.D.; JEAN-MARIE LANG, M.D.; FRANCIS OBERLING, M.D.; GEORGES MAYER, M.D.
Hereditary 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
HAUPTMANN G, MAYER S, LANG J, OBERLING F, MAYER G. Treatment of Acquired C1-Inhibitor Deficiency with Danazol. Ann Intern Med. ;87:577–578. doi: 10.7326/0003-4819-87-5-577
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Published: Ann Intern Med. 1977;87(5):577-578.
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