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一名8岁男童的小儿T细胞/组织细胞丰富型大B细胞淋巴瘤(THRLBC):病例报告

Pediatric T-Cell/Histiocyte-Rich Large B-Cell Lymphoma (THRLBC) in an 8-Year-Old Male Child: A Case Report.

作者信息

Arega Gashaw, Adam Haileyesus, Girma Alemayehu, Diida Galgaloo, Beresa Eden, Adane Leul, Negussie Michael A, Abrar Fadil Nuredin, Asefa Mesfin

机构信息

Division of Hematology and Oncology, Department of Pediatrics and Child Health, College of Health Sciences, Addis Ababa University, Addis Ababa, Ethiopia.

Department of Radiology, College of Health Sciences, Addis Ababa University, Addis Ababa, Ethiopia.

出版信息

Case Rep Oncol Med. 2025 Feb 22;2025:8869045. doi: 10.1155/crom/8869045. eCollection 2025.

DOI:10.1155/crom/8869045
PMID:40026533
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11871974/
Abstract

T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL) is a rare and aggressive subtype of diffuse large B-cell lymphoma (DLBCL) that is uncommon in children. Here, we present the case of an 8-year-old male with a 3-month history of low-grade intermittent fever, significant weight loss, loss of appetite, and progressive abdominal swelling. Examination revealed splenomegaly and a palpable midabdominal mass, with laboratory findings showing bicytopenia. Imaging demonstrated hepatosplenomegaly, diffuse hypodense liver and spleen lesions, and mesenteric and retroperitoneal lymphadenopathy. A core-needle biopsy of the mesenteric mass confirmed the diagnosis, with histopathology revealing scattered large mononuclear and binucleate cells in a background of small lymphocytes and histiocytes. Immunohistochemistry showed positivity for CD45, CD20, and EMA and negativity for CD30, CD15, and Bcl-2, excluding alternative diagnoses such as nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) and classical Hodgkin lymphoma (cHL). The patient was initially stabilized with a prephase regimen of cyclophosphamide, vincristine, and prednisone (COP), followed by induction and consolidation with R-COPADM (rituximab, cyclophosphamide, vincristine, prednisone, and methotrexate). Posttreatment imaging revealed significant resolution of lymphadenopathy and hepatosplenomegaly, with no residual or recurrent disease. At follow-up, the patient remains in clinical remission with no signs of progression. This case highlights the importance of early recognition, detailed histopathological evaluation, and the role of immunohistochemistry in accurately diagnosing THRLBCL in children, ensuring timely initiation of effective therapy and improving outcomes in this rare pediatric malignancy.

摘要

富于T细胞/组织细胞的大B细胞淋巴瘤(THRLBCL)是弥漫性大B细胞淋巴瘤(DLBCL)中一种罕见且侵袭性的亚型,在儿童中并不常见。在此,我们报告一例8岁男性患儿,有3个月的低热间歇性发热、显著体重减轻、食欲不振及进行性腹部肿胀病史。检查发现脾肿大及可触及的中腹部肿块,实验室检查结果显示全血细胞减少。影像学检查显示肝脾肿大、肝脏和脾脏弥漫性低密度病变以及肠系膜和腹膜后淋巴结肿大。肠系膜肿块的粗针活检确诊了诊断,组织病理学显示在小淋巴细胞和组织细胞背景中有散在的大单核细胞和双核细胞。免疫组化显示CD45、CD20和EMA阳性,CD30、CD15和Bcl-2阴性,排除了结节性淋巴细胞为主型霍奇金淋巴瘤(NLPHL)和经典型霍奇金淋巴瘤(cHL)等其他诊断。患者最初通过环磷酰胺、长春新碱和泼尼松(COP)的前期方案稳定病情,随后用R-COPADM(利妥昔单抗、环磷酰胺、长春新碱、泼尼松和甲氨蝶呤)进行诱导和巩固治疗。治疗后的影像学检查显示淋巴结肿大和肝脾肿大明显消退,无残留或复发病变。随访时,患者仍处于临床缓解期,无进展迹象。该病例强调了早期识别、详细的组织病理学评估以及免疫组化在准确诊断儿童THRLBCL中的重要性,确保及时开始有效的治疗并改善这种罕见儿童恶性肿瘤的预后。

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