[Immunocytoma with acquired C1-esterase inhibitor deficiency and recurrent angioneurotic edema].
Four attacks of mucosal angioneurotic oedema occurred in a 57-year-old woman, requiring tracheotomy for one episode. Decreased or non-measurable levels of complement components C1q, C-1-INH and C4 pointed to a complement consumption. The basic disease was a pleomorphic immunocytoma with the production of monoclonal IgM. Treatment with danazol, 600 mg daily, raised the serum level of C-1-INH, but not of C4.
D000799:Angioedema / Q000150:complications / Q000276:immunology* / Q000628:therapy; D000911:Antibodies, Monoclonal / Q000276:immunology; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency* / Q000276:immunology; D003181:Complement C4 / Q000172:deficiency; D003613:Danazol / Q000008:administration & dosage / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007105:Immune Complex Diseases / Q000150:complications / Q000276:immunology* / Q000628:therapy; D007121:Immunocompetence; D007075:Immunoglobulin M / Q000276:immunology; D008875:Middle Aged; D009059:Mouth Diseases / Q000276:immunology* / Q000628:therapy; D009061:Mouth Mucosa / Q000276:immunology; D012008:Recurrence; D014140:Tracheotomy
D000911:Antibodies, Monoclonal; D003174:Complement C1 Inactivator Proteins; D003181:Complement C4; D007075:Immunoglobulin M; D003613:Danazol
Immunozytom mit erworbenem C1-Esterase-Inhibitor-Mangel und rezidivierendem Angioödem.