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年轻女性小细胞碱性磷酸酶阳性间变大细胞淋巴瘤白血病表现——附 9 年生存病例报告。

Leukaemic Presentation of Small-Cell Alk-Positive Anaplastic Large Cell Lymphoma in a Young Woman-Report of a Case with 9-Year Survival.

机构信息

Departments of Pathology, Hospital Universitario Fundación Jiménez Díaz, 28040 Madrid, Spain.

Departments of Haematology, Hospital Universitario Fundación Jiménez Díaz, 28040 Madrid, Spain.

出版信息

Medicina (Kaunas). 2023 Sep 8;59(9):1628. doi: 10.3390/medicina59091628.

Abstract

Anaplastic large cell lymphoma (ALCL) with leukaemic presentation (either ab initio or along the course of the disease) has been rarely reported. Irrespective of ALK expression in the neoplastic cells, it features a dismal prognosis. We report a rare case of leukaemic, small cell variant ALK-positive ALCL with 9-year survival in a young woman who was treated upfront with corticosteroids and standard chemotherapy, and review thoroughly the previously published cases. Such an unexpected, good outcome hints at the existence of different clinical subgroups in the leukaemic variant of ALK-positive ALCL.

摘要

白血病表现型(初发或疾病进展过程中)的间变大细胞淋巴瘤(ALCL)罕见报道。不论肿瘤细胞中是否表达 ALK,均具有不良预后。我们报道了一例罕见的年轻女性白血病小细胞变异型 ALK 阳性 ALCL,患者经皮质激素和标准化疗治疗后,获得 9 年生存,我们全面复习了既往发表的病例。这种出乎意料的良好结局提示 ALK 阳性 ALCL 的白血病变异型可能存在不同的临床亚组。

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