Jabeen Kishwer, Ashraf Mohammed S, Iftikhar Sundus, Belgaumi Asim F
*Indus Children's Cancer Hospital, Karachi, Pakistan †Sidra Medical and Research Center ‡Weill Cornell Medical College - Qatar, Cornell University, Doha, Qatar.
J Pediatr Hematol Oncol. 2016 Nov;38(8):587-596. doi: 10.1097/MPH.0000000000000653.
Survival for childhood acute lymphoblastic leukemia (ALL) has improved significantly, but these benefits may not be available to many children from low and middle income countries, where reasons for treatment failure may be unique to their environment. We retrospectively reviewed data on pediatric (1 to 18 y or younger) patients with newly diagnosed ALL treated over 5 years at a children's cancer hospital in Pakistan. Patients were treated with modified Berlin-Frankfurt-Muenster -based therapy without risk stratification. There were 255 children with a median age of 7 years (mean, 7.65 y) and a male preponderance (M:F=1.6:1). 20% had T-ALL, one-third had white blood cells >50×10/L and 13.7% central nervous system disease. A majority (56.5%) was malnourished. In total, 49 (19.2%) died before the end of induction and 21 died in complete remission. Most deaths were infection-related. A total of 50 patients relapsed and 19 abandoned therapy after complete remission. Five-year overall survival is 52.9% with abandonment censored and 45.8% with abandonment as an event. Overall survival was related to socioeconomic status but not to known risk factors. The outcome of ALL at our center is suboptimal and associated with factors not commonly seen in developed countries. Special attention to early diagnosis, infection control, and parental educational are needed to improve the survival.
儿童急性淋巴细胞白血病（ALL）的生存率已有显著提高，但许多低收入和中等收入国家的儿童可能无法从中受益，这些国家治疗失败的原因可能与其所处环境独特相关。我们回顾性分析了巴基斯坦一家儿童癌症医院5年来收治的新诊断ALL儿科患者（1至18岁及以下）的数据。患者接受基于改良柏林-法兰克福-明斯特方案的治疗，未进行风险分层。共有255名儿童，中位年龄为7岁（平均7.65岁），男性居多（男:女 = 1.6:1）。20%为T-ALL，三分之一的患者白细胞计数>50×10⁹/L，13.7%有中枢神经系统疾病。大多数（56.5%）存在营养不良。共有49例（19.2%）在诱导治疗结束前死亡，21例在完全缓解期死亡。大多数死亡与感染相关。共有50例患者复发，19例在完全缓解后放弃治疗。剔除放弃治疗因素后的5年总生存率为52.9%，将放弃治疗作为事件计算的总生存率为45.8%。总生存率与社会经济状况相关，但与已知风险因素无关。我们中心ALL的治疗结果并不理想，且与发达国家常见因素不同。需要特别关注早期诊断、感染控制和家长教育，以提高生存率。
