Zonios Dimitrios I, Falloon Judith, Bennett John E, Shaw Pamela A, Chaitt Doreen, Baseler Michael W, Adelsberger Joseph W, Metcalf Julia A, Polis Michael A, Kovacs Stephen B, Kovacs Joseph A, Davey Richard T, Lane H Clifford, Masur Henry, Sereti Irini
Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892, USA.
Blood. 2008 Jul 15;112(2):287-94. doi: 10.1182/blood-2007-12-127878. Epub 2008 May 2.
Idiopathic CD4(+) lymphocytopenia (ICL) is a rare non-HIV-related syndrome with unclear natural history and prognosis. This prospective natural history cohort study describes the clinical course, CD4 T lymphocyte kinetics, outcome, and prognostic factors of ICL. Thirty-nine patients (17 men, 22 women) 25 to 85 years old with ICL were evaluated between 1992 and 2006, and 36 were followed for a median of 49.5 months. Cryptococcal and nontuberculous mycobacterial infections were the major presenting opportunistic infections. Seven patients presented with no infection. In 32, CD4 T-cell counts remained less than 300/mm(3) throughout the study period and in 7 normalized after an average of 31 months. Overall, 15 (41.6%) developed an opportunistic infection in follow-up, 5 (13.8%) of which were "AIDS-defining clinical conditions," and 4 (11.1%) developed autoimmune diseases. Seven patients died, 4 from ICL-related opportunistic infections, within 42 months after diagnosis. Immunologic analyses revealed increased activation and turnover in CD4 but not CD8 T lymphocytes. CD8 T lymphocytopenia (< 180/mm(3)) and the degree of CD4 T cell activation (measured by HLA-DR expression) at presentation were associated with adverse outcome (opportunistic infection-related death; P = .003 and .02, respectively).
特发性CD4(+)淋巴细胞减少症（ICL）是一种罕见的非HIV相关综合征，其自然史和预后尚不清楚。这项前瞻性自然史队列研究描述了ICL的临床病程、CD4 T淋巴细胞动力学、结局及预后因素。1992年至2006年间对39例年龄在25至85岁的ICL患者（17例男性，22例女性）进行了评估，其中36例患者接受了中位时间为49.5个月的随访。隐球菌和非结核分枝杆菌感染是主要的机会性感染表现形式。7例患者未出现感染。32例患者在整个研究期间CD4 T细胞计数持续低于300/mm³，7例患者平均在31个月后恢复正常。总体而言，15例（41.6%）患者在随访期间发生了机会性感染，其中5例（13.8%）为“艾滋病定义的临床病症”，4例（11.1%）发生了自身免疫性疾病。7例患者死亡，4例死于与ICL相关的机会性感染，均在诊断后42个月内。免疫分析显示，CD4 T淋巴细胞的活化和更新增加，但CD8 T淋巴细胞无此现象。CD8 T淋巴细胞减少（<180/mm³）以及初诊时CD4 T细胞的活化程度（通过HLA-DR表达测量）与不良结局（机会性感染相关死亡）相关（P值分别为0.003和0.02）。
