Abstract
Idiopathic CD4+ T lymphocytopenia (ICL) is an unusual immune defect in which there is an unexplained deficit of CD4+ T cells, leading to serious opportunistic infections. In view of the rarity of this clinical entity, we report two cases of ICL who presented with low CD4+ count or percentage and various opportunistic infections like candida, cytomegalovirus, Mycobacterium tuberculosis.
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Centers for Disease Control. Unexplained CD4+ Tlymphocyte depletion in persons without evident HIV infection- United States. MMWR Morb Mortal Wkly Rep 1992; 41: 541–545.
Wladis EJ, Kapila R, Chu DS. Idiopathic CD4+ lymphocytopenia and Sjogren syndrome. Arch Ophthalmol 2005; 123: 1012.
Inhoff O, Doerries K, Doerries R et al. Disseminated cutaneous Kaposi sarcoma and progressive multifocal leukoencephalopathy in a patient with idiopathic CD4+ T lymphocytopenia. Arch Dermatol 2007; 143: 673–675.
Smith DK, Neal JJ, Holmberg SD, Centers for Disease Control. Idiopathic CD4+ T-Lymphocytopenia Task Force. Unexplained opportunistic infections and CD4+ Tlymphocytopenia without HIV infection- an investigation of cases in the United States. N Engl J Med 1993; 328: 373–379.
Kanno H, Sasaki M, Kumagai H et al. Epstein- Barr virus-positive malignant lymphoma of salivary gland developing in an infant with selective depletion of CD4-positive lymphocytes. Leuk Lymphoma 2007; 48: 183–186.
Tanaka S, Teraguchi M, Hasui M, Taniuchi S, Ikemoto Y, Kobayashi Y. Idiopathic CD4+ T-lymphocytopenia in a boy with Down syndrome. Report of a patient and a review of the literature. Eur J Pediatr 2004; 163: 122–123.
Pasic S, Minic P, Dzudovic S, Minic A, Slavkovic B. Idiopathic CD4+ lymphocytopenia and juvenile laryngeal papillomatosis. Pediatr Pulmonol 2005; 39: 281–283.
Ohashi DK, Crane JS, Spira TJ, Courrege ML. Idiopathic CD4+ T-cell lymphocytopenia with verrucae, basal cell carcinoma and chronic tinea corporis infection. J Am Acad Dermatol 1994; 38: 889–891.
Spira TJ, Jones BM, Nicholson JKA et al. Idiopathic CD4+ T-lymphocytopenia- an analysis of five patients with unexplained opportunistic infections. N Engl J Med 1993; 328: 386–392.
Lebranchu Y, Thibault G, Degenne D et al. Abnormalities in CD4+ T lymphocyte subsets in patients with common variable immunodeficiency. Clin Immunol Immunopathol 1991; 61: 83–92.
Roger PM, Bernard-Pomier G, Counillon E et al. Overexpression of Fas/CD95 and Fas-induced apoptosis in a patient with idiopathic CD4+ T lymphocytopenia. Clin Infect Dis 1999; 28: 1012–1016.
Isgro A, Sirianni MC, Gramiccioni C et al. Idiopathic CD4+ lymphocytopenia may be due to decreased bone marrow clonogenic capability. International Arch Allergy Immunol 2005; 136: 379–384.
Früwirth M, Clodi K, Heitger A et al. Lymphocyte diversity in a 9-year-old boy with idiopathic CD4+ T-cell lymphocytopenia. International Arch Allergy Immunol 2001; 125: 80–85.
Salit RB, Hankey KG, Yi R et al. Detection of CD4+ T-cell antibodies in a patient with idiopathic CD4+ T lymphocytopenia and cryptococcal meningitis. Br J Haematol 2007; 139: 133–137.
Peterson EJ, Arska MR, Dekkar AW et al. Allogeneic bone marrow transplantation can restore CD4+ T-lymphocyte count and immune function in idiopathic CD4+ Tlymphocytopenia. Bone Marrow Transplant 1996; 18: 813–815.
Cunningham-Rundles C, Murray HW, Smith JP. Treatment of idiopathic CD4 T lymphocytopenia with IL-2. Clin Exp Immunol 1999; 116: 322–325.
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Mukherjee, A., Lodha, R. & Kabra, S.K. Idiopathic CD4+ T-cell lymphocytopenia. Indian J Pediatr 76, 430–432 (2009). https://doi.org/10.1007/s12098-009-0002-8
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DOI: https://doi.org/10.1007/s12098-009-0002-8