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Idiopathic CD4+ lymphocytopenia

Author
Karin Nielsen-Saines, MD, MPH
Section Editor
E Richard Stiehm, MD
Deputy Editor
Anna M Feldweg, MD

INTRODUCTION

Idiopathic CD4+ T cell lymphocytopenia (ICL) is a rare and heterogeneous clinical syndrome that is defined by persistent CD4+ T cell lymphopenia in the absence of infection with human immunodeficiency virus (HIV)-1 or any other cause of immunodeficiency. It is viewed as a syndrome that likely encompasses different disorders that have in common a reduction of CD4 cell numbers. Patients with ICL typically present with opportunistic infections, malignancies, or autoimmune disorders. The epidemiology, pathogenesis, clinical manifestations, diagnosis, treatment, and prognosis of ICL will be discussed in this topic review.

DEFINITION

Idiopathic CD4+ T cell lymphocytopenia (ICL) is defined by persistent CD4+ T cell lymphopenia in the absence of infection with HIV-1 or any other cause of immunodeficiency [1-5]. CD4+ T cell counts should be below 300 cells/microL or less than 20 percent of total lymphocytes on more than one occasion, usually two or three months apart. (See 'Diagnosis' below.)

BACKGROUND

Idiopathic CD4+ T cell lymphocytopenia (ICL) was first described in 1992, at the beginning of the HIV epidemic, following the recognition that acquired immunodeficiency syndrome (AIDS) was caused by the human immunodeficiency virus (HIV)-1. A subset of patients suspected of having HIV because of profound CD4+ lymphopenia had no evidence of infection. Additional immunologic defects were present in a few of these patients, including CD8+ lymphocytopenia and/or low immunoglobulin levels.

Some of these individuals also had risk factors for HIV infection. However, a thorough search for an etiologic agent in subsequent years has failed to yield a pathogen. The syndrome of ICL describes the immunologic findings in these patients.

EPIDEMIOLOGY

Idiopathic CD4+ T cell lymphocytopenia (ICL) is a rare condition that is found worldwide and has no apparent gender predilection. Most of the ICL patients originally reported were adults, although the disease has subsequently been described in a small number of children and adolescents and also in the elderly [6-10].

                                      

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Literature review current through: Nov 2016. | This topic last updated: Fri Jul 31 00:00:00 GMT 2015.
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