Lymphoma-associated paraneoplastic angioedema with normal C1-inhibitor activity: does danazol work?
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.
Gaur
S
S
Department of Medicine, Caritas St. Elizabeth's Medical Center, Tufts University School of Medicine, Boston, Massachusetts 02135, USA.
Cooley
J
J
Aish
L
L
Weinstein
R
R
D000328:Adult; D000799:Angioedema / Q000188:drug therapy* / Q000209:etiology / Q000276:immunology* / Q000473:pathology; D003174:Complement C1 Inactivator Proteins / Q000096:biosynthesis*; D003613:Danazol / Q000627:therapeutic use*; D004965:Estrogen Antagonists / Q000627:therapeutic use; D006801:Humans; D018442:Lymphoma, B-Cell, Marginal Zone / Q000150:complications* / Q000276:immunology / Q000473:pathology; D008297:Male; D013156:Splenectomy; D013160:Splenic Neoplasms / Q000150:complications* / Q000276:immunology / Q000473:pathology; D016896:Treatment Outcome
D003174:Complement C1 Inactivator Proteins; D004965:Estrogen Antagonists; D003613:Danazol