Published online Jun 16, 2021. doi: 10.12998/wjcc.v9.i17.4268
Peer-review started: December 31, 2020
First decision: February 25, 2021
Revised: March 15, 2021
Accepted: March 29, 2021
Article in press: March 29, 2021
Published online: June 16, 2021
Anaplastic 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.
We 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.
The modified LMB89 Group C regimen and ALK inhibitors are effective. Allo-HSCT should be performed after remission.
Core Tip: Anaplastic lymphoma kinase-positive (ALK+) large B-cell lymphoma (LBCL) is a rare subtype of diffuse LBCL and is particularly rare in pediatric non-Hodgkin lymphoma patients. The modified LMB89 Group C regimen is effective for ALK+LBCL. Since bone marrow failure occurs after relapse, the patient cannot tolerate intensive chemotherapy; thus, ALK inhibitors combined with low-dose chemotherapy could be considered, and there is still hope for complete remission 2. It is advised that allogeneic hematopoietic stem cell transplantation be performed as soon as possible after remission.