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

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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 plenomegaly, and progressive thrombocytopenia
Original languageEnglish
Pages (from-to)159-162
Number of pages4
JournalJournal of Clinical Immunology
Volume38
Issue number2
DOIs
Publication statusPublished - 1 Feb 2018
Externally publishedYes

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