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[原发性腔隙性淋巴瘤与HIV感染]

[Primary cavity-based lymphoma and HIV infection].

作者信息

Valencia Ortega M E, Martínez Santos P, Gómez Aguado F, Blanco Quintana F, Laguna Cuesta F, Soriano Vázquez V, González Lahoz J

机构信息

Servicio de Enfermedades Infecciosas, Instituto de Salud Carlos III, Madrid.

出版信息

Rev Clin Esp. 1999 Feb;199(2):73-7.

Abstract

BACKGROUND

Primary cavity-based lymphomas (PCBL) represent and uncommon group on non-Hodgkin lymphomas associated with AIDS. They present as malignant effusions with no bone marrow or lymph node involvement, although some cases with bone marrow infiltration at advanced stages have been reported. Tumoral cells are monoclonal and are occasionally infected with human herpesvirus type-8 (HHV-8).

PATIENTS AND METHODS

The clinical and evolutive characteristics of six HIV-positive patients with PCBL were analysed. In three of them the presence of genetic sequences of HHV-8 in peripheral blood lymphocytes and lymphomatous effusions was investigated by PCR.

RESULTS

The mean age of patients was 37 years and 5 were males. The only female patient had been drug abuser, four males were homosexuals and the other promiscuous heterosexual. The mean CD4+ lymphocyte count was 84 x 10(6)/l (range: 20-180) and all of them had been diagnosed of AIDS. The presentation forms were as pericardial effusion in one case, pleural effusion in three and tumoral ascites in two. Two of the male patients had also Kaposi sarcoma (KS). At diagnosis none of them had infiltration of the bone marrow nor lymphadenopathy. Most malignant cells had immunoblastic traits. The effusions had the characteristics of an exudate and the mean value of lactate dehydrogenase (LDH) was 5,255 IU/l (range: 1,500-11,483). In the three cases investigated there was HHV-8 DNA in the lymphocytes present in the lymphomatous effusion and in peripheral blood. The mean survival after diagnosis was 89 days (7-240). The female patient died without therapy seven days after admission and the five male patients were treated with chemotherapy with a poor response.

CONCLUSIONS

HIV-related PCBL associated or not with KA appear in severely immunodepressed patients, their behaviour is very aggressive and its clinical course fatal in a short period of time. The are often associated with KS and HHV-8 seems to be the involved causative agent.

摘要

背景

原发性腔隙性淋巴瘤(PCBL)是与艾滋病相关的非霍奇金淋巴瘤中的一个罕见类型。它们表现为恶性积液,无骨髓或淋巴结受累,不过有报道称部分晚期病例存在骨髓浸润。肿瘤细胞是单克隆性的,偶尔感染人疱疹病毒8型(HHV-8)。

患者与方法

分析了6例HIV阳性的PCBL患者的临床及病情演变特征。其中3例通过聚合酶链反应(PCR)研究外周血淋巴细胞和淋巴瘤性积液中HHV-8基因序列的存在情况。

结果

患者的平均年龄为37岁,5例为男性。唯一的女性患者曾是药物滥用者,4例男性为同性恋者而另1例为滥交的异性恋者。CD4+淋巴细胞平均计数为84×10⁶/l(范围:20 - 180),且他们均被诊断为艾滋病。表现形式为1例心包积液、3例胸腔积液和2例肿瘤性腹水。2例男性患者还患有卡波西肉瘤(KS)。诊断时他们均无骨髓浸润及淋巴结病。大多数恶性细胞具有免疫母细胞特征。积液具有渗出液的特点,乳酸脱氢酶(LDH)平均值为5255 IU/l(范围:1500 - 11483)。在研究的3例病例中,淋巴瘤性积液及外周血中的淋巴细胞存在HHV-8 DNA。诊断后的平均生存期为89天(7 - 240天)。女性患者入院7天后未接受治疗死亡,5例男性患者接受化疗,反应不佳。

结论

与KS相关或不相关的HIV相关PCBL出现在严重免疫抑制的患者中,其行为极具侵袭性,临床病程在短时间内致命。它们常与KS相关,HHV-8似乎是相关致病因子。

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