| Shuang Chen,Yongchu Huang,Yuchun Cao,Yong Zhang. Nasal-type extranodal NK/T cell lymphoma in association with hemophagocytic syndrome: a case report and literature review. Oncol Transl Med, 2022, 8: 104-108. |
| NK/T细胞淋巴瘤鼻型伴噬血细胞综合征1例报告并文献复习 |
| Nasal-type extranodal NK/T cell lymphoma in association with hemophagocytic syndrome: a case report and literature review |
| Received:November 29, 2021 Revised:April 19, 2022 |
| DOI：10.1007/s10330-021-0540-0 |
| 中文关键词: 淋巴瘤；非霍奇金；淋巴组织细胞增多症；嗜血细胞性 |
| 英文关键词: lymphoma; non-Hodgkin; lymphohistiocytosis; hemophilic |
| 基金项目:低剂量环磷酰胺有效治疗尖锐湿疣并阻止其复发的机制研究(No.81974308) |
| Author Name | Affiliation | E-mail | | Shuang Chen | Department of Dermatology and Venereology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, China | 1361421624@qq.com | | Yongchu Huang | Department of Dermatology and Venereology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, China | | | Yuchun Cao | Department of Dermatology and Venereology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, China | | | Yong Zhang* | Department of Dermatology and Venereology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, China | 61112426@qq.com |
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| 中文摘要: |
| 我们报告了一例少见的CD56阴性的NK/T细胞淋巴瘤鼻型，该患者在诊治过程中出现嗜血细胞综合征。该患者以“鼻塞3月，全身散在红斑、结节、溃疡1月”于我院就诊。我们通过对其临床表现、皮肤组织病理、免疫组化和原位杂交结果等进行分析。皮肤组织病理显示真皮全层和胶原束间中等量的异型淋巴细胞浸润。免疫组化示：CD30弥漫强（+），TIA-1(+)，CD3(2GV6) (+)，CD5部分（+），CD8部分（+），CD43 (+)，CD56 (-)，CD4 (-)，CD20(-)，PAX5(-)，PCK(-)，P63(-)，P40(-)，EGFR(-)，Ki-67(热点区LI约80%)，原位杂交:EBER-ROCH(+)。确诊为“NK/T细胞淋巴瘤鼻型”，该型淋巴瘤侵袭性强，进展快，预后极差，且早期临床表现极不典型，特别是没有出现皮疹时。分析该病的皮肤表现，对本病的早诊断、早治疗和预后有积极的作用。 |
| 英文摘要: |
| We present a rare case of nasal-type CD56-negative NK/T-cell lymphoma. The patient developed
hemophagocytic syndrome during diagnosis and treatment. The patient presented to our hospital (Tongji
Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China) with
“nasal congestion for 3 months and scattered erythema, nodules, and ulcers all over the body for 1 month.”
We analyzed clinical manifestations, skin histopathology, immunohistochemistry, and in situ hybridization
results. Histopathology of the skin revealed a moderate amount of atypical lymphocyte infiltration between
the entire dermis and collagen bundles. Immunohistochemistry showed the following: CD30 (+), TIA-1
(+), CD3(2GV6) (+), CD5 part (+), CD8 part (+), CD43 (+), CD56 (–), CD4 (–), CD20 (–), PAX5 (–), PCK
(–), P63 (–), P40 (–), EGFR (–), Ki-67 (the hot spot LI is approximately 80%), and in situ hybridization
EBER-ROCH (+). The diagnosis made was “NK/T cell lymphoma nasal type”. This type of lymphoma is
aggressive, progresses quickly, and has a poor prognosis. Early clinical manifestations are extremely
atypical, especially in the absence of rash. Analysis of the skin manifestations of the disease has a positive
effect on its early diagnosis, early treatment, and prognosis. |
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