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NK 细胞 T 细胞淋巴瘤(鼻型),一种罕见且侵袭性强的非霍奇金淋巴瘤类型:病例报告

英文原题:Natural killer cell T-cell lymphoma (nasal type), a rare and aggressive type of non-Hodgkin's lymphoma: Case report.

查看英文原题

Natural killer cell T-cell lymphoma (nasal type), a rare and aggressive type of non-Hodgkin's lymphoma: Case report.

PubMed 2023/09/04(内容时间) Radiol Case Rep

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中文摘要

鼻型结外NK/T细胞淋巴瘤是一种罕见且侵袭性强的非霍奇金淋巴瘤。在鼻腔鼻窦区域,弥漫性大B细胞淋巴瘤占主导,而鼻型NK/T细胞淋巴瘤仅占该区域非霍奇金淋巴瘤的3%–12%,且属于侵袭性较强的亚型。该病多发生于男性,中位发病年龄约50岁,预后不良,3年和5年总生存率分别为46.3%和42%。本文报告一例37岁女性,鼻腔肿块延伸至鼻咽,并伴上颈部淋巴结肿大。鼻部肿块活检提示NK/T细胞型非霍奇金淋巴瘤,但因未进行免疫组织化学检查,无法确诊。局限期疾病主要采用放化疗,完全缓解率可达50%;播散期患者则接受单纯化疗,但结局很差,5年生存率仅为10%–45%。本病例患者接受首剂化疗后,在接受第二剂前于家中死亡,体现了该病的侵袭性。通过典型影像学表现和组织学检查及时诊断,可改善结局;局限期患者中近半数有望治愈。

展开英文摘要原文

In the sinonasal tract, diffuse large B-cell lymphomas are the predominant type non-Hodgkin's lymphoma while natural killer (NK) cell T-cell lymphoma, the nasal type, constitute only 3%-12% of NHLs in this region and is the more aggressive subtype. NK cell T-cell lymphoma mostly occurs in male at the median age of 50 years and has got a poor prognosis with a 3-years overall survival (OS) of 46. 3% and a 5-years OS of 42%.

We present a case of a 37 year old lady with a mass in nasal cavity extending to the nasopharynx and upper cervical lymphadenopathy. Biopsy from the nasal mass showed features of NHL, NK cell T-cell type although immunohistochemistry was not available for the definite diagnosis. For localized diseases, chemo-radiotherapy forms the mainstay of treatment with complete remission in up to 50% of cases while chemotherapy alone is given to patients with disseminated disease with a very poor outcome (5-year survival of 10%-45% only).

In our case, the patient received first dose of chemotherapy and then died at home before receiving the second dose showing the aggressive nature of the disease. Timely diagnosis with typical imaging features and histological diagnosis can improve the outcome with complete cure in almost half of the cases with localized disease.

论文信息

作者
Kandel D、Dhakal S、Thapa S、Dhakal P、Rayamajhi S、Baniya J
第一作者单位
Department of Radiodiagnosis and Imaging, National Academy of Medical Sciences, Mahabouddha, Kathmandu, Nepal.
通讯作者单位
Department of Radiodiagnosis and Imaging, Lumbini Provincial Hospital, Butwal, Rupandehi, Nepal.
文献类型
病例报告
期刊
Radiology case reports2023 Nov
原文标识
PubMed 37701358 · DOI 10.1016/j.radcr.2023.08.033