Idiopathic CD4 Lymphocytopenia Manifesting as Refractory Genital Dysplasia

Department of Obstetrics and Gynecology and the Division of Allergy and Immunology, Department of Internal Medicine, Naval Medical Center, Portsmouth, Virginia
Obstetrics and Gynecology (Impact Factor: 5.18). 08/2013; 122(2 Pt 2):455-8. DOI: 10.1097/AOG.0b013e3182835850
Source: PubMed


Idiopathic CD4 lymphocytopenia is an immunodeficiency disorder with low absolute CD4 T-lymphocyte count with no evidence of human immunodeficiency virus or other known cause.
A 22-year-old woman presented with a high-grade Pap test result. Work-up demonstrated cervical intraepithelial neoplasia 3 and vaginal intraepithelial neoplasia 3 with extensive condyloma. She presented 6 months after her initial treatment with recurrent disease and was referred to the immunology department, where she was found to have profound lymphopenia. After further evaluation, idiopathic CD4 lymphocytopenia was diagnosed.
Idiopathic CD4 lymphocytopenia is a rare acquired immunodeficiency. Although genital dysplasia is common in young women, this case demonstrates the importance of determining other etiologies of recurrent human papillomavirus infections and possible immunodeficiencies that may affect management and outcomes.

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    ABSTRACT: Idiopathic CD4 lymphocytopenia (ICL) is defined by the reduction of the main lymphocyte subtype in peripheral blood and CD4 T cells below 300/μl in the absence of any secondary known causes of lymphopenia, including viral causes. The present review aims to state the latest available data on clinical, pathological and therapeutic aspects related to ICL, published from 1990 to 2014. The last observed clinical presentation and complications of ICL patients are described. The latest findings and possible mechanisms involved in the development of ICL features are included in the present review; however, pathogenesis of ICL has remained mainly obscured. Finally, recent therapeutic efforts considered in ICL patients are discussed. In spite of the serious complications ICL has on the patients' quality of life, data on clinical, etiopathological and therapeutic behavior for ICL are very limited. On one side, an abnormal blood cell count may be the sole presentation; however, occurrence of disseminated malignant tumors is not uncommon in patients. Recent findings highlight the role of cytokines, especially interleukin-2, on features such as phenotype severity and responsiveness of the condition to therapy. In addition, some studies have suggested that a defect in hematopoietic stem cells may be involved in disease progression, an idea that is supported by the success of bone marrow transplantation in acquiring persistent remissions in ICL patients. ICL is a hematologic condition of increasing importance due to its diverse clinical and pathological spectrum. Molecular studies have shown the presence of mutations involved in lymphocyte development as potential factors that may contribute to ICL occurrence. ICL patients could present either with common infections or really serious malignant conditions. The role of cytokines, especially interleukin-2, has emerged as one of the main possible mechanisms involved in clinical and pathological behavior of ICL. Today, the main therapeutic approaches are controlling life-threatening infections and underlying disorders along with efforts to cure ICL through rising CD4 cell counts using cytokine interventions and transplantation.
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