Sirolimus-induced improvement of immune dysregulation associated hepatosplenomegaly in common variable immunodeficiency: a case-based review.
A 50-year-old woman with common variable immunodeficiency (CVID), treated with regular intravenous immunoglobulin replacement, developed progressive hepatosplenomegaly, cytopenias, abdominal enlargement, dyspnea…
Clinical pearl
In CVID with progressive hepatosplenomegaly and cytopenias, evaluation should consider immune-mediated lymphoproliferation after excluding acute infection and overt lymphoma.