Acquired C1 esterase inhibitor deficiency in lymphomas: prevalence, symptoms, and response to treatment

Leukemia & Lymphoma
Christine BekosUlrich Jaeger

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.

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Citations

Jun 9, 2018·Allergy, Asthma, and Clinical Immunology : Official Journal of the Canadian Society of Allergy and Clinical Immunology·Arturo J BonninG Wendell Richmond
Nov 13, 2018·La Presse médicale·Laurence BouilletChantal Dumestre-Pérard
Aug 7, 2021·Clinical Immunology : the Official Journal of the Clinical Immunology Society·Yiyun Shi, Chen Wang
Nov 30, 2021·The Journal of Allergy and Clinical Immunology. in Practice·Maria L BaezaAlicia Prieto-García

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