Lee Jisoo, Park Kyeoungseo, Kim Kyoung Ha, Bang Hae In, Yoon Seug Yun, Choi In Ho
Soonchunhyang University College of Medicine.
Division of Hematology and Oncology, Department of Internal Medicine, Soonchunhyang University Seoul Hospital.
Medicine (Baltimore). 2020 Oct 16;99(42):e22657. doi: 10.1097/MD.0000000000022657.
Peripheral T cell lymphoma, not otherwise specified (PTCL-NOS) is a heterogeneous group of mature T cell lymphomas which do not correspond to any specific subtype of mature T-cell lymphoma in current classifications. Some researchers have suggested that PTCL with low Ki-67 labeling index should be classified as indolent PTCL PATIENT CONCERNS:: A 58-year old man diagnosed with alcoholic fatty liver 3 months prior complained of tenesmus and abdominal distension. Colonoscopy of the small and large intestines revealed multiple polyps, which were histologically diagnosed as lymphoid hyperplasia. One month later, he re-visited with a weight loss of 3 to 4 kg over 2 months. Radiologic examination revealed numerous small, homogenous, hypovascular lymph node enlargement in the para-aortic, mesenteric, and both inguinal areas, suggesting malignant lymphoma.
Laparoscopic biopsy of an omental lymph node was performed, which was histologically confirmed as PTCL-NOS.
The patient was administered 3 cycles of cyclophosphamide, doxorubicin, vincristine, and prednisone, but his general condition did not improve. Therefore, treatment was changed to ifosfamide, carboplatin, and etoposide -dexamethasone (4 cycles) followed by allogeneic stem cell transplantation.
Even after allogeneic stem cell transplantation, fluorodeoxyglucose uptake in his abdominal lymph nodes and small bowel in positron emission tomography- computed tomography persisted at a Deauville score of 4. The patient has been followed-up for 2 years without progression.
These indolent PTCLs histologically show diffuse infiltrated small lymphoid cells with low KI-67 labeling index and have a relatively good prognosis, although the epidemiology and pathogenesis are not fully elucidated. We report a case of indolent PTCL with cytogenetic abnormalities and poor response to chemotherapy, along with a brief review of the literature.
外周T细胞淋巴瘤,非特指型(PTCL-NOS)是一组异质性的成熟T细胞淋巴瘤,在当前分类中不符合任何特定的成熟T细胞淋巴瘤亚型。一些研究人员建议,Ki-67标记指数低的PTCL应归类为惰性PTCL。
一名58岁男性,3个月前被诊断为酒精性脂肪肝,主诉里急后重和腹胀。小肠和大肠的结肠镜检查发现多个息肉,组织学诊断为淋巴样增生。1个月后,他再次就诊,2个月内体重减轻了3至4千克。影像学检查显示腹主动脉旁、肠系膜和双侧腹股沟区有大量小的、均匀的、低血运的淋巴结肿大,提示恶性淋巴瘤。
对一个网膜淋巴结进行了腹腔镜活检,组织学确诊为PTCL-NOS。
患者接受了3个周期的环磷酰胺、多柔比星、长春新碱和泼尼松治疗,但他的一般状况没有改善。因此,治疗改为异环磷酰胺、卡铂和依托泊苷-地塞米松(4个周期),随后进行异基因干细胞移植。
即使在异基因干细胞移植后,正电子发射断层扫描-计算机断层扫描中他腹部淋巴结和小肠的氟脱氧葡萄糖摄取在Deauville评分为4时仍持续存在。该患者已随访2年无进展。
这些惰性PTCL在组织学上显示弥漫浸润的小淋巴细胞,Ki-67标记指数低,尽管流行病学和发病机制尚未完全阐明,但预后相对较好。我们报告一例伴有细胞遗传学异常且对化疗反应不佳的惰性PTCL病例,并对文献进行简要回顾。