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High prevalence of splenic marginal zone lymphoma among patients with acquired C1 inhibitor deficiency
Author(s) -
Castelli Roberto,
Wu Maddalena Alessandra,
Arquati Massimo,
Zanichelli Andrea,
Suffritti Chiara,
Rossi Davide,
Cicardi Marco
Publication year - 2016
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.13908
Subject(s) - splenic marginal zone lymphoma , medicine , lymphoma , marginal zone , immunology , splenectomy , spleen , antibody , b cell
Summary Marginal zone lymphoma represents about 10% of all non‐Hodgkin lymphomas ( NHL s). 33% of patients with acquired angioedema ( AAE ) due to acquired C1‐inhibitor (C1‐ INH ) deficiency (C1‐ INH ‐ AAE ) have or will develop NHL s. C1‐ INH ‐ AAE is a rare condition. We report the follow‐up of 72 C1‐ INH ‐ AAE patients, followed for a median of 15 years (range 1–24). Median age was 71 (range 64–79) years; median age at onset of angioedema symptoms was 57·5 (range 50–66) years and it was 63 [range 45–80) years at diagnosis]. Twenty patients were diagnosed with low‐grade non‐follicular B‐cell lymphomas (75% were splenic MZL ), one with follicular and three with high‐grade lymphomas (two diffuse large B‐cell lymphomas and one mantle cell lymphoma). Fifteen NHL s were diagnosed at onset of AAE or thereafter (3 months to 7 years), eight had already been diagnosed at onset of angioedema. Two of 24 patients remain on watchful wait. Thirthen of 24 received chemotherapy, two received rituximab. Three underwent splenectomy. All 18 patients receiving therapy for NHL experienced post‐treatment reduction in AAE symptoms. Our study suggests that clonal B‐cell proliferation is the pathology underlying AAE leading to production of C1‐ INH ‐neutralizing autoantibodies and to NHL s. The post‐germinal centre origin of NHL suggests that immune stimulation may contribute to lymphomagenesis.