title: [Angioedema caused by splenectomy with malignant lymphoma followed by multiple myeloma 7 years later].
abstract: Acquired C1-inhibitor (C1-INH) deficiency has been reported in patients with immunoglobulin abnormalities and lymphoproliferative disorders, and angioedema has appeared simultaneously with the lymphoproliferative disease. We present a 50-year-old woman with acquired C1-INH deficiency and angioedema which preceded by 7 years the diagnosis of malignant mantle cell lymphoma. During the interval she was treated with Danazole and there were no attacks of angioedema. When routine follow-up bone marrow aspiration revealed infiltration of nonspecified lymphoma cells, exploratory laparotomy and splenectomy were performed. A month later Danazol was stopped, C1-INH levels returned to normal and there were no attacks of angioedema. Mantle cell lymphoma consisting of lymphocytes with cytoplasmic IgM-lambda was diagnosed in the excised spleen but chemotherapy was not initiated. 6 months later, a second lymphoproliferative disorder, multiple myeloma IgA kappa, was diagnosed.
authors.affiliation: Institute of Allergy, Rambam Medical Center, Haifa.
authors.forename: A
authors.initials: A
authors.lastname: Shahar
authors.forename: R
authors.initials: R
authors.lastname: Sharon
authors.forename: M
authors.initials: M
authors.lastname: Lorber
authors.forename: S
authors.initials: S
authors.lastname: Pollack
mesh_terms: D000799:Angioedema / Q000188:drug therapy / Q000209:etiology*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001853:Bone Marrow / Q000473:pathology; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency*; D003613:Danazol / Q000627:therapeutic use; D004965:Estrogen Antagonists / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis; D008228:Lymphoma, Non-Hodgkin / Q000276:immunology / Q000473:pathology / Q000628:therapy*; D008875:Middle Aged; D009101:Multiple Myeloma / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D016609:Neoplasms, Second Primary / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D011183:Postoperative Complications; D013154:Spleen / Q000276:immunology / Q000473:pathology*; D013156:Splenectomy / Q000009:adverse effects
chemical_list: D003174:Complement C1 Inactivator Proteins; D004965:Estrogen Antagonists; D007070:Immunoglobulin A; D003613:Danazol