Lymphoma‐associated paraneoplastic angioedema with normal C1‐inhibitor activity: Does danazol work?

Abstract

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.

Nonallergic angioedema: role of bradykinin

“…Other diseases such as hepatocellular carcinoma and liver cirrhosis might be associated with decreased C1‐INH plasma concentration or activity, but subsequent angioedema has not been described (88, 89). There is also a report on a lymphoma‐associated angioedema with a normal plasma concentration of C1‐INH (90), and a recent characterization of a new C1‐INH mutation, leading to strong inhibition of monocyte C1‐INH secretion (91).…”

Evidence and evidence gaps of medical treatment of non-tumorous diseases of the head and neck

“…Other diseases such as hepatocellular carcinoma and liver cirrhosis can be associated with reduced C1-INH plasma concentration, but in those cases angioedema has never been described. On the other hand, one case of a lymphoma-associated angioedema is known where the C1-INH plasma concentration was regular [92]. Recently a new C1-INH mutation was described that was associated with a significantly inhibited C1-INH secretion of the monocytes [92].…”

“…On the other hand, one case of a lymphoma-associated angioedema is known where the C1-INH plasma concentration was regular [92]. Recently a new C1-INH mutation was described that was associated with a significantly inhibited C1-INH secretion of the monocytes [92]. …”

Manifestations dermatologiques des gammapathies monoclonales : apport de l’histopathologie cutanée

“…Le dosage fonctionnel du C1-INH est toujours abaissé, mais son activité antigénique est parfois conservée. Les formes acquises sont rares, associées à des syndromes lymphoprolifératifs ou des pathologies auto-immunes ou dysglobulinémiques [84][85][86][87][88][89]. Dans un contexte clinique d'oedème localisé ou généralisé peu typique, la biopsie cutanée, souvent peu spécifique, peut cependant permettre d'apporter des arguments pour d'autres diagnostics différentiels tel l'oedème de Quincke d'origine allergique, une vascularite, un oedème de la trichinose ou encore un syndrome de fuite capillaire idiopathique, mais le diagnostic repose surtout sur les paramètres biologiques et cliniques.…”