2020 CSCO Guidelines for The Diagnosis and Treatment of Lymphoma in Children and Adolescents. intercom-large cell lymphoma.

On June 21, 2020, "China Society of Clinical Oncology (CSCO) guidelines for diagnosis and treatment of childhood and adolescent lymphoma" was successfully held in the form of online live broadcast.experts and scholars in hematological oncology and pediatrics gathered online to exchange and share research results, and pay close attention to the release of the first edition of guidelines for diagnosis and treatment of lymphoma in children and adolescents.during the meeting, Professor Gao Yijin of Shanghai Children's Medical Center affiliated to Medical College of Shanghai Jiaotong University gave a detailed interpretation of the guidelines for the diagnosis and treatment of childhood and adolescent anaplastic large cell lymphoma.anaplastic large cell lymphoma (ALCL) is a mature T / NK cell lymphoma, accounting for 10-15% of non Hodgkin's lymphoma in children.1 the clinical features of 2AlCl were evaluated before treatment. General symptoms were common to 50% (fever and weight loss) extranodal lesions were common (skin, soft tissue, bone, lung, pleura and gastrointestinal diseases) bone marrow (BM) was involved ~ 5%; CNS involvement was rare and special manifestations were ① hemophagocytic syndrome (HLH) ② peripheral blood leukemia cells (manifested as severe respiratory failure) 3 the clinical staging of ALCL is based on The international staging system for childhood non Hodgkin's lymphoma staging system for ALCL: traditional morphology is still the current evaluation method for BM and CSF tumor residues; more sensitive molecular biological results are used as supporting data to record.MRI / CT was the main evaluation method; PET / CT results were recorded as supporting data.the negative value of pet > the positive value 4. The translocation gene t (2; 5) (p23; 5) involving ALK (anaplastic lymphoma kinase) could be detected in 90% of pathological diagnosis; The other 15% of the cases were rare heterotopic cases involving ALK, which could involve dusp22, p63 and other gene rearrangements. The 5-year event free survival rate of nhl-bfm90 regimen was about 76%.cumulative medication data are as follows: the data of multicenter randomized multicenter study of alcl99 is as follows (the trial involves 10 national groups or cooperative groups in 12 countries): figure patient grouping map treatment plan chart safety and survival data of alcl99 program for children and adolescents, the disease-free survival rate (DFS) of high-risk patients with ALCL in children and adolescents is about 60% - 75%.there is no data to confirm that one regimen is superior to another in the first-line treatment.for children and adolescents with ALCL, residual lesions (< 25-30% of primary lesions) are not manifestations of disease treatment failure.nhlbfm-90 is considered to be the predecessor of fre-igr-alcl99.about Changchun flower alkali (VBL): (1) FRE-IGR-ALCL99 random studies confirmed that Changchun flower alkali (VBL) can not eventually improve EFS, but can delay the recurrence time; second COG-ANHL0131 randomized study confirmed that APO based on VBL increase can only increase the toxic side effects, can not improve the survival rate; Chinese mainland currently has no legal means of VBL.the randomized study on mtxfre-igr-alcl99 confirmed that the efficacy of mtx3 arm was the same as that of mtx1 arm, but the side effects were less. 6. The main research results of progression / relapse ALCL in children are as follows: some targeted drugs, such as ALK inhibitors targeting ALK, have also appeared.ALK inhibitors used in children with ALCL are summarized as follows: in addition to ALK inhibitors, brentuximab Vedotin (BV, sgn35) can also be used in the treatment of ALCL as a new targeted drug. The relevant studies are summarized as follows: after the failure of first-line treatment, the overall survival rate of children and adolescents with advanced / recurrent ALCL is 40% - 60%; there is no standard second-line treatment scheme at present, and the overall treatment principle is to carry out autologous or allogeneic hematopoietic stem cell transplantation after the disease is relieved by various treatment methods; A variety of methods, including single drug VBL, intensive therapy, brentuximab and clazotinib, can be used alone or in combination; limited studies suggest that allogeneic hematopoietic stem cell transplantation may be superior to autologous hematopoietic stem cell transplantation.Professor Gao Yijin introduced in detail the research progress of children's ALCL treatment schemes, and compared and summarized the advantages and disadvantages of each scheme, providing reliable data support and clear guidance and suggestions for clinical treatment of children with ALCL.subsequently, Professor Zhao Donglu of Harbin Institute of Hematology and oncology, Professor Jiang Lian of the fourth hospital of Hebei Medical University, and Professor Tian Xin of Kunming children's Hospital discussed the contents of the speech, and discussed the efficacy of targeted drugs, cetylamine and other drugs in children with ALCL in the future, and the significance of peripheral blood ALK detection for detection of small residual lesions.please scan the QR code below: Stamp "read the original", and we will make progress together