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[高嗜酸性粒细胞综合征的淋巴细胞变异型]

[Lymphocytic variant of the hypereosinophilic syndrome].

作者信息

Roldán Montaud A, Citores Sánchez M J, Perales Fraile I, Masip Marzá V, Bellas Menéndez C, Vargas Núñez J A

机构信息

Servicio de Medicina Interna, Hospital Universitario Puerta de Hierro, Madrid, España.

出版信息

Rev Clin Esp. 2009 Jun;209(6):303-8. doi: 10.1016/s0014-2565(09)71478-x.

Abstract

OBJECTIVES

Hypereosinophilic syndromes (HSS) are a rare group of heterogeneous disorders characterized by prominent and persistent eosinophilia and organ dysfunction. Secondary causes of eosinophilia must be excluded. Recent advances in molecular biology and cytogenetics have permitted the characterization of different subsets of hypereosinophilic syndrome. We describe a patient with the lymphocytic variant.

PATIENT

A 46-year old male Philippine patient presented skin lesions, fever, red eyes, enlarged lymph nodes and marked eosinophilia.

RESULTS

Lymphocytic phenotyping by flow cytometry analysis was performed on peripheral blood and an aberrant population of T lymphocytes CD3-CD4+ producing interleukin 5 was found. TCR gene rearrangement using PCR amplification confirmed T cell clonality.

CONCLUSIONS

The lymphocytic variant of the hypereosinophilic syndrome is a primitive lymphocytic disorder characterized by a non-malignant T cell population expansion producing eosinophilopoietic cytokines, with an indolent clinical course but that can transform into a peripheral T lymphoma. We report the first case of such a variant published in our country and review the characteristics of this variety.

摘要

目的

高嗜酸性粒细胞综合征(HSS)是一组罕见的异质性疾病,其特征为显著且持续的嗜酸性粒细胞增多及器官功能障碍。必须排除嗜酸性粒细胞增多的继发性病因。分子生物学和细胞遗传学的最新进展使得能够对高嗜酸性粒细胞综合征的不同亚组进行特征描述。我们描述了一名淋巴细胞变异型患者。

患者

一名46岁的菲律宾男性患者出现皮肤病变、发热、眼红、淋巴结肿大及显著的嗜酸性粒细胞增多。

结果

对外周血进行流式细胞术分析进行淋巴细胞表型分析,发现一群产生白细胞介素5的异常T淋巴细胞CD3-CD4+。使用聚合酶链反应(PCR)扩增进行TCR基因重排,证实了T细胞克隆性。

结论

高嗜酸性粒细胞综合征的淋巴细胞变异型是一种原始淋巴细胞疾病,其特征为产生嗜酸性粒细胞生成细胞因子的非恶性T细胞群体扩增,临床病程进展缓慢,但可转化为外周T淋巴瘤。我们报告了我国首例此类变异型病例,并对该类型的特征进行了综述。

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