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[伴有BCL2和MYC易位的双打击滤泡性淋巴瘤]

[Double-Hit Follicular Lymphoma with BCL2 and MYC Translocations].

作者信息

Horiuchi Mirei, Fuseya Hoyuri, Tsutsumi Minako, Hayashi Yoshiki, Hagihara Kiyoyuki, Kanashima Hiroshi, Nakao Takafumi, Fukushima Yuko, Inoue Takeshi, Yamane Takahisa

机构信息

Dept. of Hematology, Osaka City General Hospital.

出版信息

Gan To Kagaku Ryoho. 2016 Sep;43(9):1135-8.

Abstract

Double-hit lymphomas are rare tumors that are defined by a chromosomal breakpoint affecting the MYC/8q24 locus in combination with another recurrent breakpoint, mainly a t(14; 18)(q32;q21)involving BCL2. We report a case of a 38-yearold woman with a 2-month history of abdominaldistention. 18F-FDG PET showed multiple positive systemic lymph nodes, positive peritoneum, and multiple positive intra-abdominal masses. Histopathology results of the cervical lymph node were compatible with double-hit follicular lymphoma(Grade 3A)because fluorescence in situ hybridization(FISH)demonstrated both MYC rearrangement and BCL2 gene fusion. She was initially started on R-CHOP(rituximab and doxorubicin, vincristine, cyclophosphamide, and prednisolone), but after one course the regimen was changed to dose-adjusted EPOCH-R(rituximab and doxorubicin, etoposide, vincristine, cyclophosphamide, and prednisolone). However, she showed no response to this chemotherapy regimen or haploidentical stem cell transplantation. The treatment strategy included salvage chemothera- py. An autologous and/or allogeneic hematopoietic transplantation is important for non-responders to DA-EPOCH-R.

摘要

双打击淋巴瘤是一种罕见肿瘤,其定义为染色体断点影响MYC/8q24基因座并伴有另一种复发性断点,主要是涉及BCL2的t(14; 18)(q32;q21)。我们报告一例38岁女性,有2个月腹胀病史。18F-FDG PET显示全身多处淋巴结阳性、腹膜阳性以及腹内多处肿块阳性。颈部淋巴结的组织病理学结果与双打击滤泡性淋巴瘤(3A级)相符,因为荧光原位杂交(FISH)显示存在MYC重排和BCL2基因融合。她最初开始接受R-CHOP(利妥昔单抗、阿霉素、长春新碱、环磷酰胺和泼尼松龙)治疗,但一个疗程后治疗方案改为剂量调整的EPOCH-R(利妥昔单抗、阿霉素、依托泊苷、长春新碱、环磷酰胺和泼尼松龙)。然而,她对该化疗方案或单倍体干细胞移植均无反应。治疗策略包括挽救性化疗。对于对DA-EPOCH-R无反应者,自体和/或异基因造血移植很重要。

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