Clinical Features and Treatment Strategies of Precursor B-cell
Lymphoblastic Leukemia with C-myC Rearrangement in Childhood
Abstract
Background: “Rare cases of Burkitt leukemia/lymphoma (BL) exhibiting a
precursor B-cell phenotype (termed herein pre-BLL) were admitted by WHO
Classification of Hematopoetic and Lymphoid Tissue, recent evidence
suggests that these neoplasms genetically and epigenetically resemble
precursor B-cell leukemia/lymphoma (pB-acute lymphoblastic
leukemia/lymphoblastic lymphoma (ALL)/LBL) rather than BL. The clinical
features and treatment of childhood pre-BLL with C-myC rearrangement are
poorly understood. Methods:The clinical features, treatment strategies
and follow-up information of 9 cases with pre-BLL diagnosed by Shanghai
Children’s Medical Center affiliated to Shanghai Jiao Tong University
School of Medicine from 2011 to 2020 were retrospectively analyzed.
Results: All the 9 cases were confirmed to be pre-BLL by flow cytometry
and fluorescence in situ hybridization, morphological classification
were L2/L3, immunophenotype was CD10, CD19 positive, CD20, TDT, CD34
selective expression, sIgM negative, Kappa and Lambda light chain
negative. Most of the pre-BLL cases were accompanied by elevated lactate
dehydrogenase (LDH), uric acid levels. 5 cases received intensive
chemotherapy with overlapping regimen, and all achieved sustained
remission except for 1 case relapse and death. Among the 4 cases who
received low-intensity chemotherapy for acute lymphoblastic leukemia, 2
cases died due to early relapse of the disease. Conclusions: Pre-BLL
cases are rare. and intensive chemotherapy treatment according to
protocols for mature B-cell NHL. Currently, the treatment strategies are
still controversial. Considering the small number of cases, multi-center
clinical studies should be actively carried out to find a standard
treatment plan.