Primary central nervous system anaplastic lymphoma kinase-positive anaplastic large cell lymphoma in children: report of one case and review of literature
10.3760/cma.j.cn115356-20201013-00242
- VernacularTitle:儿童原发中枢神经系统间变性淋巴瘤激酶阳性间变性大细胞淋巴瘤一例并文献复习
- Author:
Yongzhi ZHENG
1
;
Yi CHEN
;
Hao ZHENG
;
Jianda HU
Author Information
1. 福建医科大学附属协和医院小儿血液科 福建省血液病研究所 福建省血液病学重点实验室 福建省血液医学中心，福州 350001
- Keywords:
Lymphoma, large cell, anaplastic;
Central nervous system;
Child;
Prognosis
- From:
Journal of Leukemia & Lymphoma
2021;30(9):550-554
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the clinical features and prognosis of primary central nervous system (CNS) anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALCL) in children.Methods:The clinical data of a child with primary CNS ALK-positive ALCL in Fujian Medical University Union Hospital were retrospectively analyzed, and the relevant literature was reviewed.Results:The child went to other hospitals with headache and fever as the main symptoms. Head magnetic resonance imaging showed a right cerebellar mass, and there was no evidence of lymphoma infiltration outside the CNS before surgery. Later, cerebellar tumor resection was performed. After the surgery, through pathological examination, the child was diagnosed as ALK-positive ALCL, but did not receive chemotherapy in time. The child transferred to Fujian Medical University Union Hospital on the 27th day after surgery, and the tumor had spread to bone marrow, testis, vertebrae, etc., and the peripheral blood NPM-ALK fusion gene was positive. The child received 2 courses of chemotherapy and achieved complete remission, but eventually died of chemotherapy complications.Conclusions:Primary CNS ALK-positive ALCL is rare and easy to be misdiagnosed. The disease progresses quickly, and the overall prognosis is poor. Timely biopsy for diagnosis and early comprehensive treatment based on chemotherapy may improve the prognosis of patients.
