Tekkeşin Funda, Pınarlı Faruk Güçlü, Kaya Zühre, Oğuz Aynur, Karadeniz Ceyda, Okur Arzu, Akyürek Nalan
Department of Pediatric Oncology, Gazi University, Ankara, Turkey.
Pediatr Hematol Oncol. 2012 Nov;29(8):686-90. doi: 10.3109/08880018.2012.725238. Epub 2012 Sep 26.
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.
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。
