中国麻风皮肤病杂志 ›› 2026, Vol. 42 ›› Issue (11): 763-772.doi: 10.12144/zgmfskin202611763

• 论著 • 上一篇    下一篇

伴白血病样表现的原发皮肤非特指型外周T细胞淋巴瘤三例临床分析

关军1,易雪1,周英1,王兰兰1,王春艳2,徐金环2,王秋香1,余丹1,程辉1   

  1. 1武汉市第一医院血液内科，湖北武汉，430022；2华中科技大学同济医学院附属同济医院血液内科，湖北武汉，430030
  • 出版日期:2026-11-15 发布日期:2026-10-29

Primary cutaneous peripheral T-cell lymphoma, not otherwise specified with leukemic manifestations: a clinical analysis of 3 cases

GUAN Jun1, YI Xue1, ZHOU Ying1, WANG Lanlan1, WANG Chunyan1, XU Jinhuan2, WANG Qiuxiang1, YU Dan1, CHENG Hui1   

  1. 1 Department of Heamatology, Wuhan NO.1 Hospital, Wuhan 430022, China; 2 Department of Heamatology, Tongji Hospital, Tongji Medical College of Huazhong University of Science and Technology, Wuhan 430030, China
  • Online:2026-11-15 Published:2026-10-29

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摘要： 伴白血病样表现的原发皮肤非特指型外周T细胞淋巴瘤（primary cutaneous peripheral T-cell lymphoma, not otherwise specified, pcPTCL-NOS）呈高度侵袭性，临床预后差，其精准诊断高度依赖于组织病理学、流式细胞术及分子生物学等多维度的综合评估。本文回顾性分析2014年12月至2023年12月武汉市第一医院诊治的3例pcPTCL-NOS患者临床资料，分析其临床特征。3例患者皮损均泛发全身，呈红皮病样改变，主要表现为红斑、结节、肿块及溃疡等；外周血异常淋巴细胞比例均>20%，且均伴有皮肤、外周血、骨髓及淋巴结等多部位受累。所有患者均接受全身化疗，其中2例桥接自体造血干细胞移植。随访期内，2例死亡，1例存活。红皮病可见于多种皮肤及系统性疾病，此类患者伴发血液系统淋巴瘤受累时，临床医师应考虑pcPTCL-NOS的诊断可能。

关键词: 外周T细胞淋巴瘤, 非特指型, 皮肤淋巴瘤

Abstract: Primary cutaneous peripheral T-cell lymphoma, not otherwise specified (pcPTCLNOS) with leukemoid manifestations is a highly aggressive neoplasm associated with a poor clinical prognosis. Its accurate diagnosis relies heavily on multidimensional comprehensive evaluation including histopathology, flow cytometry and molecular biology. In this study, clinical data of three patients with pcPTCLNOS treated at Wuhan No.1 Hospital between December 2014 and December 2023 were retrospectively analyzed to explore their clinical features. All three patients presented with generalized erythrodermic skin lesions involving the wholebody, mainly manifested as erythema, nodules, masses and ulcers. The percentage of abnormal peripheralblood lymphocytes was greater than 20% in all cases, with multisite involvement of the skin, peripheral blood, bone marrow and lymph nodes. All patients received systemic chemotherapy, among whom two underwent bridging autologous hematopoietic stemcell transplantation. During the follow up period, two patients died and one survived. Erythroderma can occur in a variety of cutaneous and systemic diseases. Clinicians should raise the suspicion of pcPTCLNOS when patients with erythroderma develop hematologic lymphoma involvement.

Key words: peripheral T-cell lymphoma, not otherwise specified, cutaneous lymphoma