Subcutaneous panniculitis-like T-cell lymphoma secondary to familial hemophagocytic syndrome: report of one case and review of literature
10.3760/cma.j.cn115356-20210409-00075
- VernacularTitle:家族性噬血细胞综合征继发皮下脂膜炎样T细胞淋巴瘤一例并文献复习
- Author:
Jixin XU
1
;
Yange LI
;
Wei LIU
;
Yanna MAO
;
Yongtao DUAN
Author Information
1. 郑州大学附属儿童医院 河南省儿童医院 郑州儿童医院血液肿瘤科，郑州 450000
- Keywords:
Lymphohistiocytosis, hemophagocytic;
Child;
Panniculitis-like T-cell lymphoma
- From:
Journal of Leukemia & Lymphoma
2021;30(11):670-673
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the clinical characteristics and gene mutations of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) secondary to familial hemophagocytic syndrome (FHL).Methods:The clinical features, disease evolution, gene mutation and genetic characteristics of 1 SPTCL patient secondary to FHL in Henan Children's Hospital in June 2012 were analyzed retrospectively, and the related literatures were reviewed.Results:The UNC13D of FHL patient was homozygous mutation accompanied by STXBP2 heterozygous mutation, while that of his parents and elder brother was heterozygous mutation. After regular chemotherapy with HLH-2004 regimen, the disease relapsed 4 years later, and secondary SPTCL developed after 1 year of remission with the second chemotherapy. After giving SMILE regimen chemotherapy, allogeneic hematopoietic stem cell transplantation was performed, and now the patient had disease-free survival.Conclusions:The detection of related genes in children with hemophagocytic syndrome should be improved in time to confirm the diagnosis of primary disease. FHL can follow SPTCL, and chemotherapy combined with allogeneic hematopoietic stem cell transplantation can be the only method to cure this disease.
