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一名2.5岁儿童出现高嗜酸性粒细胞综合征的高危ALK阴性间变性大细胞淋巴瘤。

High-risk ALK negative anaplastic large-cell lymphoma presenting with hypereosinophilic syndrome in a 2.5-year-old child.

作者信息

Tekkeşin Funda, Pınarlı Faruk Güçlü, Kaya Zühre, Oğuz Aynur, Karadeniz Ceyda, Okur Arzu, Akyürek Nalan

机构信息

Department of Pediatric Oncology, Gazi University, Ankara, Turkey.

出版信息

Pediatr Hematol Oncol. 2012 Nov;29(8):686-90. doi: 10.3109/08880018.2012.725238. Epub 2012 Sep 26.

Abstract

The hypereosinophilic syndromes (HES) are characterized by prolonged nonreactive peripheral blood hypereosinophilia with tissue damage. The lymphocytic HES variant can precede malignant clonal T-cell disease in adults but it is extremely rare to be the presenting feature of lymphomas in children. Here we present a 2.5-year-old boy with HES and mediastinal T-cell anaplastic lymphoma kinase (ALK) negative systemic anaplastic large-cell lymphoma. Mature and immature eosinophils without blasts were shown on bone marrow aspiration while biopsy revealed malignant infiltration. The patient responded well to initial corticosteroid therapy, but high-risk features make a challenge of finding the cure in this extremely rare case.

摘要

嗜酸性粒细胞增多综合征(HES)的特征是外周血嗜酸性粒细胞持续增多且无反应,并伴有组织损伤。淋巴细胞性HES变体在成人中可能先于恶性克隆性T细胞疾病出现,但在儿童淋巴瘤中作为首发特征极为罕见。在此,我们报告一名2.5岁患有HES的男孩,其纵隔T细胞间变性淋巴瘤激酶(ALK)阴性的系统性间变性大细胞淋巴瘤。骨髓穿刺显示有成熟和未成熟的嗜酸性粒细胞,无原始细胞,而活检显示有恶性浸润。患者对初始皮质类固醇治疗反应良好,但高危特征给这一极其罕见病例的治愈带来了挑战。

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