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1例与嗜酸性粒细胞增多相关的T细胞淋巴增殖性疾病,对霉酚酸酯反应良好。

A case of T-cell lymphoproliferative disorder associated with hypereosinophilia with excellent response to mycophenolate mofetil.

作者信息

Hanbali Amr, Shaheen Marwan, Alfraih Feras, Al-Otaibi Wafa, El Fakih Riad, Owaidah Tarek, Ahmed Syed

机构信息

King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.

King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.

出版信息

Hematol Oncol Stem Cell Ther. 2018 Dec;11(4):241-244. doi: 10.1016/j.hemonc.2016.11.001. Epub 2017 Feb 1.

Abstract

Hypereosinophilic syndrome (HES) is a group of rare blood disorders characterized by a persistent elevation of blood eosinophil count ⩾1.5×10/L and clinical manifestations attributable to eosinophilia or tissue hypereosinophilia. Lymphocytic variant of HES (HES-L) is a known subtype according to World Health Organization classification. It is well documented in the literature that patients with HES-L are predisposed to develop T-cell lymphoma. We report a case of T-cell lymphoproliferation associated with hypereosinophilia, which has been successfully treated with mycophenolate mofetil, with resolution of skin lesions and normalization of eosinophil count and immunoglobulin E level. We believe this is a clinically relevant case since this is a rare disease with little known knowledge on its best treatment modality.

摘要

高嗜酸性粒细胞综合征(HES)是一组罕见的血液疾病,其特征为血液嗜酸性粒细胞计数持续升高⩾1.5×10⁹/L,且有嗜酸性粒细胞增多或组织嗜酸性粒细胞增多所致的临床表现。根据世界卫生组织的分类,HES的淋巴细胞变异型(HES-L)是一种已知的亚型。文献充分记载,HES-L患者易发生T细胞淋巴瘤。我们报告1例与嗜酸性粒细胞增多相关的T细胞淋巴增殖性疾病,用霉酚酸酯成功治疗,皮肤病变消退,嗜酸性粒细胞计数和免疫球蛋白E水平恢复正常。我们认为这是1例具有临床意义的病例,因为这是一种罕见疾病,对其最佳治疗方式知之甚少。

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