DOI: https://doi.org/10.22141/2224-0551.5.65.2015.74919

Clinical Case of a Rare Immunodeficiency — Idiopathic CD4+-lymphocytopenia in Pediatrician’s Practice

O.V. Urbas, M.M. Malkovych, A.B. Stefanyshyn, O.A. Holodnykh, O.V. Pastukh, I.B. Parandii, L.V. Lupuliak

Abstract


The article presents data on the prevalence, molecular and genetic aspects and potential mechanisms of idiopathic CD4+-lymphocytopenia occurrence, as well as the criteria for diagnosis in children and variants of the clinical course of this pathology. This is a rare immunity disorder with heterogeneous clinical manifestations (from asymptomatic course to severe opportunistic infections and deaths) and immunological profile. Practical observation of a clinical case of CD4+-lymphocytopenia in a child — a boy between the ages of 4 and 10 months of life has been described.


Keywords


T-lymphocytes; immunodeficiency; children

References


Centers for Disease Control [Internet]. Unexplained CD4+ T-lymphocyte depletion in persons without evident HIV infection - United States. Morbidity and Mortality Weekly Report July 30, 1992;41(30):541-545. Avialable from: http://www.cdc.gov/mmwr/preview/mmwrhtml/00017329.htm

Dawn KS, Joyce JN, Scott DH, and the Centers for Disease Control Idiopathic CD4+ T-Lymphocytopenia Task Force. Unexplained Opportunistic Infections and CD4+ T-Lymphocytopenia without HIV Infection - An Investigation of Cases in the United States. N Engl J Med. 1993;328:373-379. doi:10.1056/NEJM19930211328060.

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. doi:10.1016/S0190-9622(94)70253-5.

Freier S, Kerem E, Dranitzki Z, et al. Hereditary CD4+ T lymphocytopenia. Arch Dis Child. 1998;78(4):371-372. doi:10.1136/adc.78.4.371.

Hubert P, Bergeron F, Ferreira V, et al. Defective p56Lck activity in T-cells from an adult patient with idiopathic CD4+- lymphocytopenia. Int Immunol. 2000;12(4):449-457. doi:10.1093/intimm/12.4.449.

Isgr? A, Sirianni MC, Gramiccioni C, Mezzaroma I, Fantauzzi A, Aiuti F. Idiopathic CD4+ lymphocytopenia may be due to decreased bone marrow clonogenic capability. Int Arch Allergy Immunol. 2005;136(4):379-384. doi:10.1159/000084258.

Mukherjee A, Lodha R, Kabra SK. Idiopathic CD4+ T-cell Lymphocytopenia. Indian J Pediatr. 2009;76(4):430-432. doi:10.1007/s12098-009-0002-8.

Li FY, Chaigne-Delalande B, Kanellopoulou C, et al. Second messenger role for Mg2+ revealed by human T-cell immunodeficiency. Nature. 2011;475(7357):471-476. doi:10.1038/nature10246.

Kuijpers TW, Ijspeert H, van Leeuwen EM, et al. Idiopathic CD4+ T lymphopenia without autoimmunity or granulomatous disease in the slipstream of RAG mutations. Blood J. 2011;117(22):5892-5896. doi:10.1182/blood-2011-01-329052.

Zonios D, Sheikh V, Sereti I. Idiopathic CD4 lymphocytopenia: a case of missing, wandering or ineffective T cells. Arthritis Res Ther. 2012;14:222. doi: 10.1186/ar4027.

Tariq K, Guzman N. Idiopathic CD4+ T-cell Lymphocytopenia (ICL): an Extremely Uncommon Presentation of ICL with PCP in an HIV Negative Patient. JSM Clin Case Rep. [Internet] 2014;2(2):1030. Avialable from: http://www.jscimedcentral.com/CaseReports/casereports-2-1030.pdf




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