伴有异常T细胞、7号染色体三体及血清TARC水平升高的嗜酸性粒细胞增多症。

Hypereosinophilia with abnormal T cells, trisomy 7 and elevated TARC serum level.

作者信息

Roumier A S, Grardel N, Laï J L, Becqueriaux I, Ghomari K, de Lavareille A, Roufosse F, Prin L, Capron M

机构信息

INSERM U547, Institut Pasteur, 1 rue du Professeur Calmette, BP 245, 59019 LILLE Cedex, FRANCE.

出版信息

Haematologica. 2003 Jul;88(7):ECR24.

PMID:12857571

Abstract

The idiopathic hypereosinophilic syndrome (HES) is a rare heterogeneous disorder, characterized by persistent blood eosinophilia with possible organ involvement. We describe here the case of a 20-year-old atopic male presenting chronic hypereosinophilia and eczema since childhood. Biological findings included hypereosinophilia (9.5 x 10(9)/L), hyperlymphocytosis (10.9 x 10(9)/L), polyclonal hypergammaglobulinemia and elevated IgE serum level. Flow cytometric analysis of blood lymphoid cells showed a population of CD2+CD3-CD4+TCRab-TCRgd- lymphocytes. These cells displayed a Th0/Th2 cytokine profile, and a clonal TCR rearrangement pattern. A high serum TARC level was observed. Karyotype studies on blood stimulated culture or lymph nodes revealed a cellular hyperdiploïd clone 47, XY, +7. To our knowledge, this chromosomal aberration has never been reported in such case.

摘要

特发性嗜酸性粒细胞增多综合征（HES）是一种罕见的异质性疾病，其特征为持续性血液嗜酸性粒细胞增多，并可能累及器官。我们在此描述一名20岁的特应性男性病例，该患者自童年起就出现慢性嗜酸性粒细胞增多和湿疹。生物学检查结果包括嗜酸性粒细胞增多（9.5×10⁹/L）、淋巴细胞增多（10.9×10⁹/L）、多克隆高球蛋白血症以及血清IgE水平升高。对血液淋巴细胞进行流式细胞术分析显示存在一群CD2⁺CD3⁻CD4⁺TCRab⁻TCRgd⁻淋巴细胞。这些细胞呈现Th0/Th2细胞因子谱和克隆性TCR重排模式。观察到血清TARC水平较高。对血液刺激培养物或淋巴结进行的核型研究显示存在一个细胞超二倍体克隆47，XY，+7。据我们所知，这种染色体畸变在该病例中从未有过报道。