[Clinical features and therapeutic effect of 38 children with anaplastic large cell lymphoma].
OBJECTIVE
To analyze the clinical features and prognostic factors of children's anaplastic large cell lymphoma (ALCL), summarize the therapeutic effect and toxicities.


METHODS
A total of 38 ALCL patients admitted to Beijing Children's Hospital from Jan. 2003 to Apr. 2010 were treated with BCH-ALCL-2003 regimen (modified from HK-ALCL-2000).


RESULTS
Thirty-four cases were ALK(+), male:female ratio = 2.16:1. The median age was 9 years; 86.8% had B symptoms. 94.7% evolved to Stage III and IV on admission. The median follow-up duration was 48 months (12 to 99 months). Median event-free survival (EFS) time was 43 months. Thirty-four patients (89.5%) achieved a remission. The disease relapsed in 3 patients within 20 months after diagnosis. Estimated 4-year EFS was (81.2 ± 6.4)%, estimated 4-year overall survival (OS) rate was (86.4 ± 5.7)%. Univariate analysis indicated that the unfavorable prognostic factors included: more than 3 extra nodal involvement, hepatosplenomegaly (> 3 cm), elevated lactate dehydrogenase (LDH), stage IV, hemophagocytosis in bone marrow, and age < 3 years. The major toxicity was myelosuppression and mucositis. no chemotherapy related death occurred.


CONCLUSIONS
(1) Childhood ALCL patients often have B symptoms and extranodal involvement. (2) In the study, therapeutic effects was good. The disease relapsed mostly within the first 2 years, maintenance therapy with vinblastine is necessary. (3) The regimen is safe to patients.
Center of Hematology, Beijing Children's Hospital Affiliated to Capital Medical University, Beijing 100045, China.
Jing
J
Yang
Ling
L
Jin
Hu-yong
HY
Zheng
Rui
R
Zhang
Yan-long
YL
Duan
Shuang
S
Huang
Chun-ju
CJ
Zhou
Yong-hong
YH
Zhang
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D017728:Lymphoma, Large-Cell, Anaplastic / Q000175:diagnosis* / Q000628:therapy*; D008297:Male; D011379:Prognosis; D016896:Treatment Outcome