Idiopathic CD4 lymphopenia (ICL) is a rare clinical syndrome characterized by CD4+ T cell count of less than 300 cells/mm3, or less than 20% of total T cells, without evidence of HIV infection or other immunodeficiency. Patients with cellular immunodeficiency are at higher risk for invasive fungal infection. We present the first reported case of ICL associated with disseminated coccidioidomycosis.
A 44 year old woman with a ten year history of leukopenia, recalcitrant human papilloma virus (HPV), pharyngeal abscess, and disseminated coccidioidomycosis, presented for evaluation. There was no history of recurrent sinopulmonary, gastroinestinal or cutaneous infection, autoimmune disorder or malignancy.
White blood cell count was 2800 with an absolute lymphocyte count of 400. Absolute CD4+ and CD8+ T cell counts were decreased at 23 and 46, respectively. HIV viral load and antibody levels were undetectable. NK cell count was normal but function was depressed. Normal studies included quantitative immunoglobulins, pneumococcal titers, complement function, neutrophil oxidase burst, HTLV I/II Ab, and bone marrow biopsy. No monoclonal populations were detected. Lymphocyte antigen and mitogen panel showed absent lymphocyte responses to Candida. CSF analysis detected coccidioidomycosis antibodies.
Our patient had persistently decreased CD4+ T-lymphocytes in the absence of other etiologies consistent with a diagnosis of ICL. She had low CD8+ T-lymphocytes, not routinely observed, conferring higher mortality rates. Lifelong prophylactic fluconazole, trimethoprim-sulfamethoxazole, and azithromycin were prescribed. Therapies with IL-2 or HSCT have been proposed. The pathogenesis and management of ICL are not well defined and require further research.
- AAAAI 2015 (71st)