Idiopathic CD4 + T cell Lymphocytopenia (ICL): an Extremely Uncommon Presentation of ICL with PCP in an HIV Negative Patient

At the beginning of the HIV/AIDS epidemic in the 1980s, the medical community learned about a subset of patient population who presented with opportunistic infections, had severely depressed CD4 T cell counts, but to everyone’s surprise these patients did not have HIV infection and there was no other explanation to account for their immunodeficiency [15]. Emergence of these anomalous findings lead to a review of 230,179 cases in the Center for Disease Control and Prevention (CDC) AIDS Reporting System from 1980s to the early 1990s and found 47 cases of Idiopathic CD4+ T cell lymphocytopenia (ICL) [6]. To our knowledge these 47 cases represent the largest pool of ICL patients and who were then studied prospectively between 1992 through 2006 to define the natural history of the disease [6-7]. Most of our understanding of the rare ICL syndrome comes from the findings obtained from these 47 patients [7].