中华皮肤科杂志 ›› 2023, Vol. 56 ›› Issue (2): 105-111.doi: 10.35541/cjd.20220048

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皮肤T细胞淋巴瘤继发噬血细胞性淋巴组织细胞增多症7例临床分析

关军1    帅华洲1    王兰兰1    王春艳2    程平1    胡彬3    易雪1    邹亮1    程辉1   

  1. 1武汉市第一医院血液内科，武汉  430022；2华中科技大学同济医学院附属同济医院血液内科，武汉  430074；3武汉市第一医院皮肤科，武汉  430022
  • 收稿日期:2022-01-20 修回日期:2022-08-02 发布日期:2023-02-01
  • 通讯作者: 程辉 E-mail:whsyyyxyk@163.com
  • 基金资助:
    武汉市医学科研项目（WX21C24）

Clinical analysis of seven cases of hemophagocytic lymphohistiocytosis secondary to cutaneous T-cell lymphoma

Guan Jun1, Shuai Huazhou1, Wang Lanlan1, Wang Chunyan2, Cheng Ping1, Hu Bin3, Yi Xue1, Zou Liang1, Cheng Hui1   

  1. 1Department of Hematology, Wuhan No.1 Hospital, Wuhan 430022, China; 2Department of Hematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Hubei 430074, China; 3Department of Dermatology, Wuhan No.1 Hospital, Wuhan 430022, China
  • Received:2022-01-20 Revised:2022-08-02 Published:2023-02-01
  • Contact: Cheng Hui E-mail:whsyyyxyk@163.com
  • Supported by:
    Wuhan Medical Scientific Research Project（WX21C24）

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摘要： 【摘要】 目的 探讨皮肤T细胞淋巴瘤（CTCL）合并继发性噬血细胞性淋巴组织细胞增多症（sHLH）的临床及实验室特征。方法 回顾性分析2016年6月至2021年10月武汉市第一医院血液内科收治的CTCL并拟诊sHLH患者，按照HLH-2004标准和HScore评分对患者进行评估。结果 7例CTCL患者确诊合并sHLH，分别为2例原发性皮肤γδT细胞淋巴瘤（PC-GDTCL），3例皮肤结外NK/T细胞淋巴瘤（C-ENKTCL），2例原发皮肤间变大细胞淋巴瘤（PC-ALCL）。7例患者均接受化疗，6例死亡，确诊CTCL合并sHLH后生存期14 ~ 60 d （中位生存期26.5 d）。2例检出HLH相关基因突变（PRF1、LYST突变），2例PC-GDTCL患者分别检出淋巴瘤相关基因突变（KRAS、KMT2D和JAK3 、SAMHD1突变）。结论 CTCL合并sHLH临床进展迅速，需早期诊断及干预，初诊及病情进展时行骨穿检查及淋巴瘤、HLH相关基因突变检测有助于早期诊断。

关键词: 淋巴瘤, T细胞, 皮肤, 淋巴组织细胞增多症, 嗜血细胞性, 淋巴瘤, 原发性皮肤间变性大细胞, 淋巴瘤, 结外NK-T细胞

Abstract: 【Abstract】 Objective To investigate clinical and laboratory characteristics of secondary hemophagocytic lymphohistiocytosis （sHLH） associated with secondary cutaneous T-cell lymphoma （CTCL）. Methods CTCL patients with clinically suspected sHLH were collected from Department of Hematology, Wuhan No.1 Hospital from January 2016 to October 2021, and were evaluated according to the HLH-2004 diagnostic criteria and HScore. Results Seven CTCL patients were confirmedly diagnosed with sHLH, including 2 with primary cutaneous γδT-cell lymphoma （PC-GDTCL）, 3 with cutaneous extranodal natural killer/T-cell lymphoma （C-ENKTCL）, and 2 with primary cutaneous anaplastic large cell lymphoma （PC-ALCL）. All the 7 patients received chemotherapy, but 6 died finally, and the median overall survival duration was 26.5 days （range: 14 - 60 days） after the confirmed diagnosis of CTCL complicated by sHLH. HLH-related gene mutations， which were located in the PRF1 and LYST genes, were identified in 2 patients; lymphoma-related gene mutations were identified in the KRAS and KMT2D genes in 1 PC-GDTCL patient,and in the JAK3 and SAMHD1 genes in another PC-GDTCL patient. Conclusions CTCL complicated by sHLH usually progresses rapidly, so early diagnosis and treatment are needed. Bone marrow biopsy and mutation screening of lymphoma- and HLH-related genes at initial diagnosis and during disease progression may facilitate early diagnosis.

Key words: Lymphoma, T-cell, cutaneous, Lymphohistiocytosis, hemophagocytic, Lymphoma, primary cutaneous anaplastic large cell, Lymphoma, extranodal NK-T-cell

引用本文

关军 帅华洲 王兰兰 王春艳 程平 胡彬 易雪 邹亮 程辉. 皮肤T细胞淋巴瘤继发噬血细胞性淋巴组织细胞增多症7例临床分析[J]. 中华皮肤科杂志, 2023,56(2):105-111. doi:10.35541/cjd.20220048

Guan Jun, Shuai Huazhou, Wang Lanlan, Wang Chunyan, Cheng Ping, Hu Bin, Yi Xue, Zou Liang, Cheng Hui. Clinical analysis of seven cases of hemophagocytic lymphohistiocytosis secondary to cutaneous T-cell lymphoma[J]. Chinese Journal of Dermatology, 2023, 56(2): 105-111.doi:10.35541/cjd.20220048