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两岁儿童肝脾γδ T 细胞淋巴瘤:一例病例报告

英文原题:Gamma-Delta Hepatosplenic T-Cell Lymphoma in a Two-Year-Old: A Case Report.

查看英文原题

Gamma-Delta Hepatosplenic T-Cell Lymphoma in a Two-Year-Old: A Case Report.

PubMed 2026/01/23(内容时间) Cureus

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中文摘要

γδ肝脾T细胞淋巴瘤(HSTCL)是一种罕见的侵袭性恶性肿瘤,在幼儿中极为罕见。其常表现为非特异性症状,导致诊断延迟。我们描述了一名既往健康的26个月女童,出现每日发热、体重下降、食欲减退及进行性下肢无力,最终导致无法行走。体格检查显示明显肝脾肿大,无淋巴结肿大,实验室检查显示淋巴细胞性白细胞增多伴逐渐出现的血细胞减少。骨髓评估发现克隆性γδ T细胞群,肝活检证实肝窦浸润,从而确立γδ HSTCL的诊断。其临床过程以对多种诱导、巩固及靶向治疗反应不佳为特征。她表现为持续性微小残留病(MRD),需要反复升级治疗及大量支持治疗。本病例说明了儿童γδ HSTCL的诊断困难及治疗耐药性。早期识别、分子特征分析以及及时考虑可能的外周血干细胞移植对于改善这一罕见且高度侵袭性儿童淋巴瘤的预后至关重要。

展开英文摘要原文

Gamma-delta (γδ) hepatosplenic T-cell lymphoma (HSTCL) is a rare, aggressive malignancy that is exceptionally uncommon in young children. It often presents with nonspecific symptoms that delay diagnosis.

We describe a previously healthy 26-month-old girl who developed daily fevers, weight loss, decreased appetite, and progressive lower-extremity weakness leading to loss of ambulation. Examination revealed marked hepatosplenomegaly without lymphadenopathy, and laboratory studies showed lymphocytic leukocytosis with evolving cytopenias. Bone marrow evaluation identified a clonal γδ T-cell population, and liver biopsy confirmed sinusoidal infiltration, establishing the diagnosis of γδ HSTCL.

Her clinical course was characterized by poor response to multiple induction, consolidation, and targeted therapies. She presented with persistent minimal residual disease (MRD), requiring repeated treatment escalation and substantial supportive care. This case illustrates the diagnostic difficulty and therapeutic resistance of pediatric γδ HSTCL. Early recognition, molecular characterization, and timely consideration of possible stem cell transplant are essential to improving outcomes in this rare and highly aggressive pediatric lymphoma.

论文信息

作者
Mohammed H、Khan A、Lin F、Mourad A、Butala M
第一作者单位
Medicine, Lake Erie College of Osteopathic Medicine, Bradenton, USA.United States
通讯作者单位
Pediatrics, Ascension St. Vincents, Jacksonville, USA.United States
文献类型
病例报告
期刊
Cureus2026 Jan
原文标识
PubMed 41732614 · DOI 10.7759/cureus.102138