[Clinical features and prognosis of children with lymphoblastic lymphoma].
OBJECTIVE
To evaluate the clinical characteristics of childhood lymphoblastic lymphoma (LBL) and therapeutic efficacy of BCH-LBL-2003 regimen modified from BFM-90 protocol. The drug-related toxicities and prognostic factors were explored at the same time.


METHODS
From Janurary 2003 to December 2009, 112 newly diagnosed LBL patients at the Hematology Center of Beijing Children's Hospital were enrolled in this study. The patients were treated with modified BFM-90-LBL protocol.


RESULTS
At a median follow-up of 29 months (1 to 90 months), the patients were evaluated on day 33 and at the end of induction therapy. The bone marrow complete remission (CR) rates were 96.4% and 100%, respectively. Meanwhile, the complete remission (CR) rates of tumor were 77.7% and 94.5%, and the partial remission (PR) rates were 22.3% and 5.5%, respectively. The overall response rate was 100%. The 3-year overall survival (OS) rate was 89.1% and 5-year OS was 87.0%. The 3-year event-free survival (EFS) was 85.4% and 5-year EFS was 83.3%. Eleven cases relapsed during the treatment (4 BM relapses, 3 CNS recurrences, 3 primary site and 1 lymph node of neck and BM). Eleven patients died (3 died of infection and 8 died of progressive disease after relapse). All patients experienced grade 3-4 hematological toxicity. Univariate analysis indicated that lack of CR at the end of induction therapy, immunophenotype, bulky tumor and course of disease had prognostic significance.


CONCLUSIONS
Lymphoblastic lymphoma in childhood and adolescence is highly aggressive. BCH-LBL-2003 protocol is very effective. The treatment-associated side effects were tolerable. Patients who didn't get CR at the end of induction therapy, with T-cell immunophenotype, with bulky disease and the course of disease less than 30 days may have a poor prognosis.
Hematology Center, Beijing Children's Hospital Affiliated to Capital Medical University, Beijing 100045, China.
Ling
L
Jin
Rui
R
Zhang
Shuang
S
Huang
Jing
J
Yang
Yan-Long
YL
Duan
Yong-Hong
YH
Zhang
D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001853:Bone Marrow / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D009367:Neoplasm Staging; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy* / Q000473:pathology*; D012074:Remission Induction; D015996:Survival Rate