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Reversible Suppression of Lymphoproliferation and Thrombocytopenia with Rapamycin in a Patient with Common Variable Immunodeficiency

Abstract

Splenomegaly is a well-recognized complication in patients with common variable immunodeficiency (CVID), particularly those with an abnormal accumulation of mature CD21lo B cells in peripheral blood [1]. In CVID, splenomegaly is associated with hypersplenism, autoimmune cytopenias, and secondary lymphoma [2]. Patients with suspected lymphoma and refractory cytopenias may require splenectomy despite intensive treatment with steroids and rituximab, and this places them at increased risk of overwhelming post-splenectomy infections [3, 4]. We describe a patient with late-onset primary antibody deficiency, lymphoproliferative disease with generalized lymphadenopathy and massive splenomegaly, and progressive thrombocytopenia. The patient had expanded mature CD21lo B cells and activated effector memory CD4+ and CD8+ T cells, and overactive mTOR signaling. Treatment with rapamycin corrected the thrombocytopenia and reversed the lymphoproliferation.

Type Journal
ISBN 1573-2592 (Electronic) 0271-9142 (Linking)
Authors Deenick, E. K.; Morey, A.; Danta, M.; Emmett, L.; Fay, K.; Gracie, G.; Ma, C. S.; Macintosh, R.; Smith, Sabc; Sasson, S. C.; Sewell, W. A.; Cowley, M.; Tangye, S. G.; Kelleher, A. D.; Circa; Phan, T. G.
Responsible Garvan Author Dr Elissa Deenick
Publisher Name JOURNAL OF CLINICAL IMMUNOLOGY
Published Date 2018-02-28
Published Volume 38
Published Issue 2
Published Pages 159-162
Status Published in-print
DOI 10.1007/s10875-018-0477-z
URL link to publisher's version https://www.ncbi.nlm.nih.gov/pubmed/29350338