← 返回

原发性皮肤γ-δ T 细胞淋巴瘤伴噬血细胞性淋巴组织细胞增生症一例年轻波利尼西亚男性患者

英文原题:Primary Cutaneous Gamma-Delta T-Cell Lymphoma Presenting With Hemophagocytic Lymphohistiocytosis in a Young Polynesian Male.

查看英文原题

Primary Cutaneous Gamma-Delta T-Cell Lymphoma Presenting With Hemophagocytic Lymphohistiocytosis in a Young Polynesian Male.

PubMed 2025/05/23(内容时间) Case Rep Hematol Q4 · IF 0.7(JCR 2025)

分数与星级只用于站内排序 —— 不代表疗效、安全性或个人适用性。

中文摘要

原发性皮肤γδT细胞淋巴瘤(PCGD-TCL)是一种非常罕见的皮肤T细胞淋巴瘤亚型。我们报告了一例年轻的波利尼西亚男性病例,表现为发热和腹壁皮疹,并重点介绍了导致PCGD-TCL诊断的检查过程。由于PCGD-TCL罕见且可模仿其他疾病,其诊断需要高度警惕,并且可能具有挑战性。噬血细胞性淋巴组织细胞增生症(HLH)可与PCGD-TCL同时发生,并可能是更具侵袭性疾病的标志。目前尚无明确的治疗指南,但最常见的治疗方法是基于蒽环类药物的多药联合化疗,随后进行异基因干细胞移植。靶向治疗也正被越来越多地使用。预后仍然较差,5年生存率< 20%,尤其是在更具侵袭性的疾病中。我们重点说明了该患者的人口学特征与已发表文献的差异,并讨论了诊断评估和治疗中的一些独特细节,尤其是在合并HLH的情况下。

展开英文摘要原文

Primary cutaneous gamma-delta T-cell lymphoma (PCGD-TCL) is a very rare subtype of cutaneous T-cell lymphoma.

We report the case of a young Polynesian male who presented with fever and an abdominal wall rash and highlight the workup leading to the diagnosis of PCGD-TCL. As PCGD-TCL is rare and mimics other medical conditions, its diagnosis requires a high index of suspicion and can be challenging. Hemophagocytic lymphohistiocytosis (HLH) occurs with PCGD-TCL and can be a marker of more invasive disease.

There are no well-defined treatment guidelines, but the most common treatment approach is anthracycline-based multiagent chemotherapy followed by allogeneic stem cell transplant. Targeted therapies are being increasingly used as well. Prognosis remains poor and 5-year survival is < 20%, particularly in more invasive disease.

We highlight how this patient's demographic varies from the published literature and discuss some unique particulars of the diagnostic evaluation and treatment, especially in the presence of concurrent HLH.

论文信息

作者
Arabi S、Vardell V、Hanley T、Florell S、Halwani A、Lim M
第一作者单位
Department of Internal Medicine, University of Utah, Salt Lake City, Utah, USA.United States
通讯作者单位
Division of Hematology and Hematologic Malignancies, Huntsman Cancer Institute, University of Utah, Salt Lake City, Utah, USA.United States
文献类型
病例报告
期刊
Case reports in hematology2025
原文标识
PubMed 40452922 · DOI 10.1155/crh/8582804