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[慢性瘙痒性皮疹揭示血管免疫母细胞性T细胞淋巴瘤]

[Chronic prurigo revealing an angioimmunoblastic T cell lymphoma].

作者信息

Khaled Aida, Sfia Mehdi, Fazaa Becima, Kourda Nadia, Zermani Rachida, Baccouche Kamel, Ben Jilani Sara, Kamoun Mohamed Ridha

机构信息

Service de Dermatologie, Hôpital Charles Nicolle, Tunis, Tunisie.

出版信息

Tunis Med. 2009 Aug;87(8):534-7.

Abstract

BACKGROUND

Cutaneous manifestations in angio-immunoblastic T cell lymphoma (AITL) can be seen in almost 50% of patients. They are especially represented by maculo-papular pseudotoxic exanthema. The other manifestations, like nodular prurigo are rarely observed. We report a case of AITL diagnosed after an etiologic screening for chronic prurigo.

AIM

The objective of our work is to stress on the possibility to diagnose a hemopathy in etiologic screening of chronic pruritus.

CASE REPORT

A 45-year-old patient presented a chronic pruritus of 18 months associated with general manifestations (fever, night sweating and weight loss). Physical examination showed diffuse adenomegalies. On histology, the lymph nodes were composed of polymorphous lymphoid proliferation made of middle to large sized cells with clusters of epithelioid cells and post-capillary veinules hyperplasia. Immunohistochemical study showed T cell phenotype (CD3+). Large cells were CD30+. Latent Protein of EBV virus was not expressed. Molecular biological analysis of a lymph node showed a T cell clonal proliferation. Cutaneous biopsy showed a little dermic perivascular lymphocytic inflammatory infiltrate. The diagnosis of angio-immunoblastic T cell lymphoma was made. The abdomino-pelvian CT scanner showed multiple inter-aortico-cave lymph nodes and a splenomegaly. A polychemotherapy was initiated.

CONCLUSION

In front of chronic prurigo with general manifestations, a careful etiologic screening should be done to detect internal disorders especially malignant hemopathies.

摘要

背景

血管免疫母细胞性T细胞淋巴瘤(AITL)患者中近50%可见皮肤表现。其典型表现为斑丘疹样假中毒性疹。其他表现,如结节性痒疹则较为罕见。我们报告1例在对慢性痒疹进行病因筛查后确诊的AITL病例。

目的

我们工作的目的是强调在慢性瘙痒的病因筛查中诊断血液病的可能性。

病例报告

1例45岁患者出现18个月的慢性瘙痒,并伴有全身症状(发热、盗汗和体重减轻)。体格检查发现全身淋巴结肿大。组织学检查显示,淋巴结由多形性淋巴细胞增殖组成,细胞为中到大型,伴有上皮样细胞簇和毛细血管后小静脉增生。免疫组化研究显示为T细胞表型(CD3+)。大细胞CD30+。EB病毒潜伏蛋白未表达。对一个淋巴结进行分子生物学分析显示T细胞克隆性增殖。皮肤活检显示真皮血管周围有少量淋巴细胞炎性浸润。诊断为血管免疫母细胞性T细胞淋巴瘤。腹盆腔CT扫描显示多个主动脉腔静脉间淋巴结及脾肿大。开始进行多药化疗。

结论

对于伴有全身症状的慢性痒疹,应进行仔细的病因筛查以发现潜在疾病,尤其是恶性血液病。

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