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.

journal_name

Leuk Lymphoma

journal_title

Leukemia & lymphoma

authors

Bekos C,Perkmann T,Krauth M,Raderer M,Lechner K,Jaeger U

doi

10.3109/10428194.2015.1131273

subject

Has Abstract

pub_date

2016-09-01 00:00:00

pages

2033-6

issue

9

eissn

1042-8194

issn

1029-2403

journal_volume

57

pub_type

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