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特发性CD4 + T淋巴细胞减少症——5例不明原因机会性感染患者的分析

Idiopathic CD4+ T-lymphocytopenia--an analysis of five patients with unexplained opportunistic infections.

作者信息

Spira T J, Jones B M, Nicholson J K, Lal R B, Rowe T, Mawle A C, Lauter C B, Shulman J A, Monson R A

机构信息

Immunology Branch, Centers for Disease Control and Prevention, Atlanta, GA 30333.

出版信息

N Engl J Med. 1993 Feb 11;328(6):386-92. doi: 10.1056/NEJM199302113280603.

DOI:10.1056/NEJM199302113280603
PMID:8093635
Abstract

BACKGROUND

Although patients with idiopathic CD4+ T-lymphocytopenia and serious opportunistic infections have been described previously, the clinical and immunologic features of this condition have not been well defined.

METHODS

We studied in detail five patients with idiopathic CD4+ T-lymphocytopenia. The studies included serologic testing, culture, and polymerase chain reaction for the human immunodeficiency virus (HIV) types 1 and 2, serologic testing for the human T-cell lymphotropic virus (HTLV) types I and II, lymphocyte phenotyping, immunoglobulin quantitation, and lymphocyte-transformation assays, as well as attempts to isolate a retroviral agent. The results were compared with those in HIV-infected persons matched for CD4+ T-cell counts and with those in normal controls. We also studied the spouses of patients and the blood donors for one patient.

RESULTS

In these five patients, there was no evidence of either HIV or HTLV infection. All the patients had both low percentages and low counts of CD4+ T cells, with relative increases in percentages, but not counts, of CD8+ cells. Numbers of B cells and natural killer cells were generally normal. As compared with HIV-infected persons, our patients had lower percentages and counts of CD8+ cells and more lymphopenia. CD4+ counts were relatively stable over time. Instead of the high immunoglobulin levels seen in HIV infection, these patients had normal or slightly low levels of immunoglobulins. The lymphocyte-transformation response to mitogens and antigens was depressed. Results in spouses and blood donors were normal.

CONCLUSIONS

Idiopathic CD4+ T-lymphocytopenia differs from HIV infection in its immunologic characteristics and in its apparent lack of progression over time. Nothing about the immunologic or viral-culture studies performed in these patients or about their family members or blood donors suggests that a transmissible agent causes this condition.

摘要

背景

尽管先前已描述过患有特发性CD4+ T淋巴细胞减少症并伴有严重机会性感染的患者,但这种疾病的临床和免疫学特征尚未明确界定。

方法

我们详细研究了5例特发性CD4+ T淋巴细胞减少症患者。研究包括对1型和2型人类免疫缺陷病毒(HIV)进行血清学检测、培养及聚合酶链反应,对I型和II型人类T细胞嗜淋巴细胞病毒(HTLV)进行血清学检测,淋巴细胞表型分析,免疫球蛋白定量,淋巴细胞转化试验,以及尝试分离逆转录病毒病原体。将结果与CD4+ T细胞计数相匹配的HIV感染者及正常对照者的结果进行比较。我们还研究了患者的配偶以及其中1例患者的献血者。

结果

在这5例患者中,没有证据表明感染了HIV或HTLV。所有患者的CD4+ T细胞百分比和计数均较低,CD8+细胞百分比相对增加,但计数未增加。B细胞和自然杀伤细胞数量通常正常。与HIV感染者相比,我们的患者CD8+细胞百分比和计数较低,淋巴细胞减少更明显。CD4+计数随时间相对稳定。与HIV感染中所见的高免疫球蛋白水平不同,这些患者的免疫球蛋白水平正常或略低。对有丝分裂原和抗原的淋巴细胞转化反应受到抑制。配偶和献血者的检测结果正常。

结论

特发性CD4+ T淋巴细胞减少症在免疫学特征以及明显缺乏随时间进展方面与HIV感染不同。对这些患者及其家庭成员或献血者进行的免疫学或病毒培养研究,均未表明有可传播病原体导致这种疾病。

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