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噬血细胞性淋巴组织细胞增生症合并2例儿童淋巴细胞消减型经典型霍奇金淋巴瘤

Hemophagocytic Lymphohistiocytosis Associated With 2 Cases of Pediatric Lymphocyte-depleted Classic Hodgkin Lymphoma.

作者信息

Cader Fathima Z, Colmenero Isabel, Mussai Francis

机构信息

Birmingham Children's Hospital, Steelhouse Lane, Birmingham, UK.

出版信息

J Pediatr Hematol Oncol. 2019 Jul;41(5):e341-e345. doi: 10.1097/MPH.0000000000001398.

DOI:10.1097/MPH.0000000000001398
PMID:30601404
Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare and often fatal syndrome of abnormal T-cell activation and cytokine production, which can be familial or secondary in nature. Although HLH can occur concomitantly with lymphomas, the development of HLH alongside Hodgkin lymphoma in children is unusual. Here we report the diagnostic evaluation and clinical course of 2 pediatric cases of HLH secondary to lymphocyte-depleted classic Hodgkin lymphoma. These cases highlight the need to be vigilant for this rare presentation and the difficulties in managing these patients.

摘要

噬血细胞性淋巴组织细胞增生症(HLH)是一种罕见且常致命的综合征,其特征为T细胞异常激活和细胞因子产生,可呈家族性或继发性。尽管HLH可与淋巴瘤同时发生,但儿童霍奇金淋巴瘤并发HLH的情况并不常见。本文报告了2例淋巴细胞消减型经典霍奇金淋巴瘤继发HLH的儿科病例的诊断评估及临床过程。这些病例凸显了对这种罕见表现保持警惕的必要性以及管理这些患者的困难。

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