title: Acquired C1 esterase inhibitor deficiency in lymphomas: prevalence, symptoms, and response to treatment.
abstract: We retrospectively studied the prevalence of C1 esterase inhibitor (C1 INH) deficiency in 131 patients with various lymphomas. We determined C1 INH activity, C1 INH antigen, and C4 concentration at diagnosis and after chemotherapy. In follicular lymphoma (FL), diffuse large B-cell lymphoma (DLBCL) and chronic lymphocytic leukemia (CLL) consecutive patients were studied. In these entities, the prevalence of C1 INH deficiency was 10.2% in DLBCL, 4.1% in CLL, and 0% in FL and Hodgkin lymphoma. In indolent lymphomas, we identified only single cases of C1 INH deficiency, predominantly in splenic marginal zone lymphomas (SMZL) (four cases). Only three patients were symptomatic while the majority (11 cases) was asymptomatic. In DLBCL patients who were successfully treated with chemotherapy, complete normalization of C1 INH activity and C4 was observed. In contrast, C1 INH deficiency remained in SMZL patients after splenectomy. We conclude that C1 INH deficiency in lymphomas is frequently asymptomatic and responsive to immunochemotherapy.
authors.affiliation: a Department of Medicine I, Division of Hematology and Hemostaseology , Medical University of Vienna , Vienna , Austria ;
authors.forename: Christine
authors.initials: C
authors.lastname: Bekos
authors.affiliation: b Clinical Institute of Laboratory Medicine and Clinical Chemistry, Medical University of Vienna , Vienna , Austria ;
authors.forename: Thomas
authors.initials: T
authors.lastname: Perkmann
authors.affiliation: a Department of Medicine I, Division of Hematology and Hemostaseology , Medical University of Vienna , Vienna , Austria ;
authors.forename: Maria
authors.initials: M
authors.lastname: Krauth
authors.affiliation: c Department of Medicine I, Division of Clinical Oncology , Comprehensive Cancer Center, Medical University of Vienna , Vienna , Austria.
authors.forename: Markus
authors.initials: M
authors.lastname: Raderer
authors.affiliation: a Department of Medicine I, Division of Hematology and Hemostaseology , Medical University of Vienna , Vienna , Austria ;
authors.forename: Klaus
authors.initials: K
authors.lastname: Lechner
authors.affiliation: a Department of Medicine I, Division of Hematology and Hemostaseology , Medical University of Vienna , Vienna , Austria ;
authors.forename: Ulrich
authors.initials: U
authors.lastname: Jaeger
mesh_terms: D000328:Adult; D000368:Aged; D054179:Angioedemas, Hereditary / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy; D015415:Biomarkers; D003131:Combined Modality Therapy; D003172:Complement C1 / Q000276:immunology; D003174:Complement C1 Inactivator Proteins / Q000235:genetics / Q000276:immunology / Q000378:metabolism; D003181:Complement C4 / Q000276:immunology; D005260:Female; D006801:Humans; D007167:Immunotherapy; D008223:Lymphoma / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D015995:Prevalence; D013156:Splenectomy; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D015415:Biomarkers; D003172:Complement C1; D003174:Complement C1 Inactivator Proteins; D003181:Complement C4
keywords: Allergy and hypersensitivity; autoimmunity: responses to self-antigen; chemotherapeutic approaches; lymphoma and Hodgkin lymphoma