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Diagnosis and treatment of pediatric anaplastic lymphoma kinase-positive large B-cell lymphoma: A case report
Authors:Meng Zhang  Ling Jin  Yan-Long Duan  Jing Yang  Shuang Huang  Mei Jin  Guang-Hua Zhu  Chao Gao  Yi Liu  Nan Zhang  Chun-Ju Zhou  Zi-Fen Gao  Qin-Long Zheng  Dong Chen  Yong-Hong Zhang
Abstract:
BACKGROUNDAnaplastic lymphoma kinase-positive (ALK+) large B-cell lymphoma (LBCL) is a rare type of lymphoma with high invasiveness and rapid progression. It occurs in all age groups, but is extremely rare in children. The lesions mainly involve the lymph nodes and may present with extra-nodal involvement. Response to conventional chemotherapies and local radiotherapy is poor, with a 5-year overall survival of less than 40%. Recently, the use of ALK inhibitors for the treatment of this disease has been reported.CASE SUMMARYWe present a case of a 12-year-old boy diagnosed with ALK+LBCL. The patient had a 2-mo medical history of a calvarial mass, extensive systemic involvement, and positive bone marrow clathrin heavy chain (CLTC)-ALK fusion gene. Complete remission 1 (CR1) was achieved using the modified LMB89 Group C regimen followed by autologous stem cell transplantation. The patient relapsed 3 mo later. He then achieved CR2 with three short courses of chemotherapy (COP, reduced-dose ICE, low-dose Ara-c+VP16) and continuous alectinib targeted therapy. Afterward, allogeneic hematopoietic stem cell transplantation (allo-HSCT) was performed. At 16 mo after the allo-HSCT, the patient was still in CR2.CONCLUSIONThe modified LMB89 Group C regimen and ALK inhibitors are effective. Allo-HSCT should be performed after remission.
Keywords:Pediatric   Anaplastic lymphoma kinase-positive large B-cell lymphoma   CLTC-ALK   PGS1-CLTC   Hematopoietic stem cell transplantation   Alectinib   Case report
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