Primary leptomeningeal lymphoma in a patient with concomitant CD4+ lymphocytopenia

Abstract

Idiopathic CD4+ lymphocytopenia (ICL) is a rare disorder in which patients have mild and/or severe opportunistic infections or maybe without symptoms. The etiology is currently unknown. Diagnosis is made by excluding retroviral infections (human immunodeficiency virus-1 or -2, human T cell lymphotropic virus-1 or -2) or other known causes of immunosuppression. To provide a case report of a patient with possible ICL who presented with a rare form of primary non-Hodgkin lymphoma (NHL) of the central nervous system (CNS). Review of the literature has identified only five other patients with NHL and ICL; however, none of these had a CNS lymphoma. We describe a patient with possible ICL, and address links between lymphopenia and lymphoproliferative disorders. Although not uncommon for patients infected with human immunodeficiency virus to develop CNS NHL, this is the first case of a possible ICL patient with such a lymphoma. This case revisits an important relationship between lymphopenia and lymphoproliferative disorders.