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Lymphoma‐associated paraneoplastic angioedema with normal C1‐inhibitor activity: Does danazol work?
Author(s) -
Gaur S.,
Cooley J.,
Aish L.,
Weinstein R.
Publication year - 2004
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.20195
Subject(s) - danazol , angioedema , medicine , c1 inhibitor , lymphoma , dermatology , pathology , endometriosis
A patient with splenic marginal zone lymphoma presented with severe, symptomatic acquired angioedema. Unlike previously reported cases, his serum levels of complement and C1 inhibitor (C1‐INH) were not decreased. Nonetheless, he responded clinically to treatment with an attenuated androgen and, after therapeutic splenectomy, has been maintained asymptomatic without androgen therapy for 5 years. Thus stimulation of C1‐INH synthesis may overcome paraneoplastic angioedema in patients with lymphoproliferative disorders despite the absence of typical evidence for a quantitative or qualitative defect in C1‐INH. Androgens should not be withheld despite a normal level of C1‐INH and complement in symptomatic patients. Am. J. Hematol. 77:296–298, 2004. © 2004 Wiley‐Liss, Inc.