EB virus-associated ALK-negative anaplastic large cell lymphoma complicated with hemophagocytic syndrome in children: report of 1 case and review of literature
10.3760/cma.j.cn115356-20240523-00071
- VernacularTitle:儿童EB病毒相关ALK阴性间变大细胞淋巴瘤合并噬血细胞综合征1例并文献复习
- Author:
Xiaohong ZHANG
1
;
Wenling GUO
;
Wenge HAO
;
Ru ZHANG
;
Hua JIANG
Author Information
1. 广州医科大学附属广州市妇女儿童医疗中心血液肿瘤科，广州 510700
- Keywords:
Anaplastic large cell lymphoma;
Hemophagocytic syndrome;
Child;
EB virus
- From:
Journal of Leukemia & Lymphoma
2025;34(4):218-221
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To improve the understanding of anaplastic lymphoma kinase (ALK)-negative anaplastic large cell lymphoma complicated with hemophagocytic syndrome (HPS) in children.Methods:A retrospective analysis was conducted on the diagnosis and treatment of a case of EB virus-associated ALK-negative anaplastic large cell lymphoma in a child with HPS as the clinical manifestation at Guangzhou Women and Children's Medical Center Affiliated to Guangzhou Medical University in December 2019, and literature review was conducted.Results:The patient was an 8-year-old boy who was admitted with facial yellowing and recurrent fever. After comprehensive examination, he was diagnosed with HPS. After 2 weeks of chemotherapy according to the hemophagocytic lymphohistiocytosis (HLH)-1994 regimen, lymph node biopsy was performed. Immunohistochemistry showed that CD30, CD5, CD4, CD7, EMA, TIA-1, and VIM were positive, ALK, CD2, CD3, CD8, CD117, CD20, INI-1, CD68, MyoD1, Myogenin, Desmin, Langerin, SALL4, CD56, GramB, and CK were negative, LCA was weakly positive, TFE3 was partially weakly positive, and Ki-67 positivity index was 90%. The clonality assay for TCRD gene rearrangement was positive. The supplementary diagnosis was ALK-negative anaplastic large cell lymphoma, and the clinical risk stratification was classified as high-risk group. After 2 courses of chemotherapy with the South China Children's Cancer Group-non-Hodgkin lymphoma 2017 regimen (SCCCG-NHL-2017), he was evaluated as complete remission (CR), and after 6 courses, he was still evaluated as CR. The patient received autologous hematopoietic stem cell transplantation. The patient was followed up until May 2024, his survival status was good.Conclusions:EB virus-associated ALK-negative anaplastic large cell lymphoma complicated with HPS in children is rare, chemotherapy combined with autologous hematopoietic stem cell transplantation is a feasible treatment option.
