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一名特发性骨髓纤维化患者在脾切除术后发生孤立性粒细胞肉瘤,伴有der(1;7)(q10;p10),最终转化为急性髓系白血病。

Idiopathic myelofibrosis developing isolated granulocytic sarcoma with der (1;7)(q10; p10) after splenectomy and finally transforming to acute myelogenous leukemia.

作者信息

Kasahara S, Tsurumi H, Hara T, Goto H, Moriwaki H

机构信息

First Department of Internal Medicine, Gifu University School of Medicine, 40 Tsukasa-machi, Gifu 500-8705, Japan.

出版信息

Leuk Lymphoma. 2000 Oct;39(3-4):427-33. doi: 10.3109/10428190009065844.

Abstract

A 47-year-old female with idiopathic myelofibrosis developed isolated granulocytic sarcoma with der (1; 7)(q10; p10) after splenectomy, followed by acute myelogenous leukemia. The patient had myelofibrosis since 22 years of age, received splenectomy at 47 years, and developed isolated submandibular granulocytic sarcoma, 8 months later. Although the initial tumor disappeared after irradiation, recurrent tumors selectively appeared in the areas of operative scars. She subsequently developed blastic transformation with der (1; 7)(q10; p10), and the blasts were refractory to different chemotherapy. This case is very rare in the following aspects: the onset of myelofibrosis occurred at a relatively young age; isolated granulocytic sarcoma after splenectomy preceded the transformation to acute leukemia; and the subcutaneous tumors developed in areas of operative scars.

摘要

一名47岁的特发性骨髓纤维化女性患者在脾切除术后发生了伴有der(1;7)(q10;p10)的孤立性粒细胞肉瘤,随后发展为急性髓系白血病。该患者自22岁起患有骨髓纤维化,47岁时接受了脾切除术,8个月后出现孤立性下颌下粒细胞肉瘤。尽管初始肿瘤在放疗后消失,但复发性肿瘤选择性地出现在手术瘢痕部位。她随后发生了伴有der(1;7)(q10;p10)的原始细胞转化,且原始细胞对不同的化疗均耐药。该病例在以下方面非常罕见:骨髓纤维化发病年龄相对较轻;脾切除术后的孤立性粒细胞肉瘤先于向急性白血病的转化;皮下肿瘤在手术瘢痕部位发生。

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