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Primary leptomeningeal lymphoma in a patient with concomitant CD4+ lymphocytopenia
Paula J Busse1, Charlotte Cunningham-Rundles
1Division of Clinical Immunology, Mount Sinai Medical Center, New York, New York 10029, USA. paula.busse@mssm.edu
Background:
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.
Objective:
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.
Results:
We describe a patient with possible ICL, and address links between lymphopenia and lymphoproliferative disorders.
Conclusions:
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.