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特发性CD4+ T淋巴细胞减少症——无HIV感染证据的免疫缺陷。

Idiopathic CD4+ T-lymphocytopenia--immunodeficiency without evidence of HIV infection.

作者信息

Ho D D, Cao Y, Zhu T, Farthing C, Wang N, Gu G, Schooley R T, Daar E S

机构信息

Aaron Diamond AIDS Research Center, New York.

出版信息

N Engl J Med. 1993 Feb 11;328(6):380-5. doi: 10.1056/NEJM199302113280602.

DOI:10.1056/NEJM199302113280602
PMID:8093634
Abstract

BACKGROUND

The human immunodeficiency virus (HIV), the etiologic agent of the acquired immunodeficiency syndrome (AIDS), infects and depletes CD4+ T lymphocytes. Recently, patients have been described with profound CD4+ T-lymphocytopenia but without evident HIV infection, a condition now termed idiopathic CD4+ T-lymphocytopenia, and a national surveillance network has been set up to investigate such cases.

METHODS

We studied 12 patients with CD4+ T-lymphocytopenia who were referred to us from three U.S. cities. Blood samples were tested for HIV with specific antibody assays, viral cultures, and polymerase-chain-reaction (PCR) techniques.

RESULTS

The patients (10 men and 2 women) ranged in age from 30 to 69 years. Eight had risk factors for HIV infection. The clinical manifestations were heterogeneous: five patients had opportunistic infections, five had syndromes of unknown cause, and two had no symptoms. Two patients died from acute complications of their immunodeficiency. The patients' lowest CD4+ lymphocyte counts ranged from 3 to 308 per cubic millimeter (mean, 149). Three patients had complete or partial spontaneous reversal of the CD4+ T-lymphocytopenia. Concomitant CD8+ T-lymphocytopenia was noted in three patients, and abnormal immunoglobulin levels were found in five. Multiple virologic studies by serologic testing, culture, and PCR were completely negative for HIV in all patients.

CONCLUSIONS

Our 12 patients with idiopathic CD4+ T-lymphocytopenia appear to be epidemiologically, clinically, and immunologically heterogeneous. It is unclear whether this syndrome is new, transmissible, or acquired. Many of the clinical and immunologic features are distinct from those found in AIDS, and our extensive virologic studies found no evidence of HIV infection. The cause of this condition remains unknown.

摘要

背景

人类免疫缺陷病毒(HIV)是获得性免疫缺陷综合征(AIDS)的病原体,可感染并消耗CD4+ T淋巴细胞。最近,有患者被描述为存在严重的CD4+ T淋巴细胞减少,但无明显的HIV感染,这种情况现在被称为特发性CD4+ T淋巴细胞减少症,并且已经建立了一个全国性监测网络来调查此类病例。

方法

我们研究了从美国三个城市转诊至我们这里的12例CD4+ T淋巴细胞减少患者。采用特异性抗体检测、病毒培养和聚合酶链反应(PCR)技术对血样进行HIV检测。

结果

患者(10名男性和2名女性)年龄在30至69岁之间。8名患者有HIV感染的危险因素。临床表现各异:5名患者有机会性感染,5名患者有病因不明的综合征,2名患者无症状。2名患者死于免疫缺陷的急性并发症。患者的最低CD4+淋巴细胞计数范围为每立方毫米3至308个(平均为149个)。3名患者的CD4+ T淋巴细胞减少症出现了完全或部分自发逆转。3名患者伴有CD8+ T淋巴细胞减少,5名患者发现免疫球蛋白水平异常。通过血清学检测、培养和PCR进行的多项病毒学研究在所有患者中均显示HIV检测结果为阴性。

结论

我们的12例特发性CD4+ T淋巴细胞减少患者在流行病学、临床和免疫学方面似乎存在异质性。尚不清楚这种综合征是否是新出现的、可传播的或后天获得的。许多临床和免疫学特征与AIDS不同,并且我们广泛的病毒学研究未发现HIV感染的证据。这种情况的病因仍然未知。

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