Duncan R A, von Reyn C F, Alliegro G M, Toossi Z, Sugar A M, Levitz S M
Evans Memorial Department of Clinical Research, Boston City Hospital, MA 02118.
N Engl J Med. 1993 Feb 11;328(6):393-8. doi: 10.1056/NEJM199302113280604.
We describe four patients without major risk factors for human immunodeficiency virus (HIV) infection, each of whom presented with severe opportunistic infections and was found to have idiopathic CD4+ T-lymphocytopenia. We performed assays to detect the presence of retroviruses and undertook immunophenotyping of subgroups of peripheral-blood lymphocytes.
The opportunistic infections at presentation included Pneumocystis carinii pneumonia, cryptococcal meningitis (two patients, one with concurrent pulmonary tuberculosis), and histoplasma-induced brain abscess. During 10 to 68 months of observation, none of the four patients had evidence of infection with HIV type 1 or 2 or human T-cell lymphotropic virus type I or II on the basis of epidemiologic, serologic, or polymerase-chain-reaction studies or culture, nor was there any detectable reverse transcriptase activity. Although all the patients had severe, persistent CD4+ T-lymphocytopenia (range, 12 to 293 cells per cubic millimeter), the CD4+ cell count progressively declined in only one and was accompanied by multiple opportunistic infections. All four patients had significantly reduced numbers of circulating CD8+ T cells, natural killer cells, or B cells (or all three).
These four patients had idiopathic CD4+ T-lymphocytopenia with opportunistic infections but no evidence of HIV infection. Instead of the progressive, selective depletion of CD4+ T cells characteristic of HIV infection, some patients with idiopathic immunodeficiency have stable CD4+ cell counts accompanied by reductions in the levels of several other lymphocyte subgroups.
我们描述了4例无人类免疫缺陷病毒（HIV）感染主要危险因素的患者，他们均出现严重的机会性感染，且被发现患有特发性CD4⁺ T淋巴细胞减少症。我们进行了检测逆转录病毒存在的试验，并对外周血淋巴细胞亚群进行了免疫表型分析。
初诊时的机会性感染包括卡氏肺孢子虫肺炎、隐球菌性脑膜炎（2例患者，其中1例同时合并肺结核）以及组织胞浆菌引起的脑脓肿。在10至68个月的观察期内，基于流行病学、血清学、聚合酶链反应研究或培养，这4例患者均无感染1型或2型HIV或I型或II型人类嗜T细胞病毒的证据，也未检测到任何逆转录酶活性。尽管所有患者均有严重且持续的CD4⁺ T淋巴细胞减少（范围为每立方毫米12至293个细胞），但仅1例患者的CD4⁺细胞计数逐渐下降，并伴有多种机会性感染。所有4例患者的循环CD8⁺ T细胞、自然杀伤细胞或B细胞（或三者）数量均显著减少。
这4例患者患有伴有机会性感染的特发性CD4⁺ T淋巴细胞减少症，但无HIV感染证据。与HIV感染特征性的CD4⁺ T细胞进行性、选择性耗竭不同，一些特发性免疫缺陷患者的CD4⁺细胞计数稳定，但伴有其他几种淋巴细胞亚群水平降低。
