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作 者:Jing Yang Jun Li Wei-Yue Gu Ling Jin Yan-Long Duan Shuang Huang Meng Zhang Xi-Si Wang Yi Liu Chun-Ju Zhou Chao Gao Hu-Yong Zheng Yong-Hong Zhang
机构地区:[1]Beijing Key Laboratory of Pediatric Hematology Oncology,National Discipline of Pediatrics,Ministry of Education,MOE Key Laboratory of Major Diseases in Children/Hematology Oncology Center,Beijing Children's Hospital,Capital Medical University,National Center for Children’s Health,Beijing 100045,China [2]Chigene(Beijing)Translational Medical Research Center Co.,Ltd.,Beijing 101111,China
出 处:《World Journal of Clinical Cases》2020年第9期1685-1692,共8页世界临床病例杂志
基 金:Supported by the Special Fund of the Pediatric Medical Coordinated Development Center of Beijing Hospitals Authority,No.XTZD20180204。
摘 要:BACKGROUND The aberrant expression of the anaplastic lymphoma kinase(ALK)gene in ALKpositive(ALK+)anaplastic large cell lymphoma(ALCL)is usually due to t(2;5)/NPM-ALK.However,rarely,aberrant ALK expression can also result from a rearrangement of the ALK gene with various partner genes.Central nervous system(CNS)metastasis is very rare in ALK+ALCL.Patients with CNS involvement show an inferior prognosis.CASE SUMMARY Here,we present the case of an 8-year-old girl diagnosed with ALK+ALCL.She presented with fever,skin nodules,leg swelling,and abdominal pain over the preceding 6 mo.She had extensive involvement and showed an extraordinary rare translocation,t(2;17)/CLTC-ALK,as demonstrated by RNA-seq.She underwent chemotherapy as per ALCL99,followed by vinblastine(VBL)maintenance treatment,and achieved complete remission.However,she developed CNS relapse during VBL monotherapy.The patient achieved a durable second remission with high-dose chemotherapy(including methotrexate 8 g/m2)and continuous treatment with alectinib and VBL.CONCLUSION Alectinib showed significant and durable CNS effects in this patient.However,more cases are needed to prove the efficacy and safety of alectinib for pediatric ALK+ALCL patients.
关 键 词:ANAPLASTIC LYMPHOMA PEDIATRIC CLTC/ALK Central nervous system Alectinib Case report
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