多学科联合诊治1例NK/T细胞淋巴瘤相关噬血细胞综合征

唐佳宁; 蒋欣怡; 潘华雄; 陈万新; 汪晶; 钟刚; 蔡丽; 尹艳雪; 崔国惠; 张璐

doi:10.13201/j.issn.1004-2806.2024.09.008

多学科联合诊治1例NK/T细胞淋巴瘤相关噬血细胞综合征

- 1.
  华中科技大学同济医学院附属协和医院血液病研究所(武汉，430022)
- 2.
  华中科技大学同济医学院附属协和医院病理科
- 3.
  华中科技大学同济医学院附属协和医院放射科
- 4.
  华中科技大学同济医学院附属协和医院耳鼻咽喉科

详细信息

通讯作者: 张璐，E-mail：luluzhang103@163.com

中图分类号: R733.4

收稿日期: 2024-06-04

刊出日期: 2024-09-01

Multidisciplinary treatment of NK/T-cell lymphoma associated hemophagocytic lymphohistiocytosis

- 1.
  Institute of Hematology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China
- 2.
  Department of Pathology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology
- 3.
  Department of Radiology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology
- 4.
  Department of Otolaryngology Head & Neck Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology

More Information

Corresponding author: ZHANG Lu, E-mail: luluzhang103@163.com

Received Date: 04 June 2024

Publish Date: 01 September 2024

摘要

摘要: 本文介绍1例合并噬血细胞综合征的鼻型结外NK/T细胞淋巴瘤(extranodal NK/T-cell lymphoma-hemophagocytic lymphohistiocytosis，ENKTCL-HLH)的多学科联合诊治(multidisciplinary team，MDT)过程。患者入院前后持续高热，经验性抗感染药物治疗效果不佳，合并全血细胞减少、鼻腔肿物和EBV感染，来我院就诊时已处于HLH的晚期。虽然临床上高度怀疑原发于鼻腔的ENKTCL，但患者生命体征不稳定，存在难以纠正的凝血功能异常和重度血小板减少，进一步行鼻咽活检明确继发HLH原因的风险极高。经过我院MDT讨论，已有证据难以区分EBV-HLH和ENKTCL，决定给予DEP方案(脂质体多柔比星+依托泊苷+甲泼尼龙)继续控制HLH，待生命体征稳定后再行病理活检。鼻腔活检病理确诊ENKTCL(鼻型)，DEP方案治疗2周后患者症状反复，考虑HLH控制不佳。结合患者病理结果，调整为L-MEP方案(米托蒽醌脂质体+依托泊苷+甲泼尼龙+培门冬酶)后再次诱导HLH达到缓解，后立即续贯针对原发病ENKTCL的P-GemOx方案(培门冬酶+吉西他滨+奥沙利铂)化疗，最终缓解后出院。该患者诊治过程体现了MDT在ENKTCL-HLH等病程复杂凶险疾病中的重要价值。此外，本例通过L-MEP方案成功治疗难治性淋巴瘤相关HLH，进一步证实米托蒽醌脂质体联合依托泊苷和甲泼尼龙治疗成人HLH的安全性和有效性，为相关临床研究奠定基础。
- 噬血细胞综合征 /
- 结外NK/T细胞淋巴瘤 /
- L-MEP方案 /
- 多学科联合诊治
Abstract: This article presents the multidisciplinary team(MDT) approach to the diagnosis and treatment of a case involving nasal extranodal NK/T-cell lymphoma(ENKTCL) complicated with hemophagocytic lymphohistiocytosis(HLH). The patient, admitted with persistent fever and refractory to standard antimicrobials, exhibited pancytopenia, a nasal mass, and evidence of EBV infection. By the time of presentation at our facility, the patient had progressed to the late stages of HLH. Despite strong clinical indicators suggestive of primary nasal ENKTCL, the patient's hemodynamic instability, coupled with coagulopathy and severe thrombocytopenia, posed an exceedingly high risk for a nasopharyngeal biopsy to ascertain the etiology of secondary HLH. Following comprehensive deliberation by our MDT, it was determined that the existing evidence was inconclusive to distinguish between EBV-HLH and ENKTCL. Consequently, the decision was made to administer the DEP regimen to manage HLH, with the intention of conducting a pathological biopsy once the patient's condition was stabilized. Histopathological examination of the nasal biopsy confirmed the diagnosis of ENKTCL(nasal type). After the two-week course of DEP therapy, the patient experienced a relapse of symptoms, indicating that the HLH was refractory to the treatment. In light of the histopathological findings, the therapeutic strategy was revised to the L-MEP regimen, which successfully induced remission of HLH. Thereafter, the patient was promptly initiated on P-GemOx chemotherapy targeting the underlying ENKTCL. The patient was discharged following clinical remission. This case exemplifies the pivotal role of MDT in managing aggressive conditions such as ENKTCL-HLH. Furthermore, the successful resolution of refractory lymphoma associated HLH with the L-MEP regimen implies the therapeutic potential of mitoxantrone liposomal in combination with etoposide and methylprednisolone for adult HLH, contributing to the evidence for future clinical trials.
- hemophagocytic lymphohistiocytosis /
- extranodal natural killer/T-cell lymphoma /
- L-MEP regimen /
- multidisciplinary team

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