Fadoo Zehra, Nisar Imran, Yousuf Fatimah, Lakhani Laila Saleem, Ashraf Shamvil, Imam Uzma, Zaheer Junaid, Naqvi Ahmed, Belgaumi Asim
Department of Pediatrics and Child Health and Department of Oncology, Aga Khan University, Karachi, Pakistan.
Medical Student, Aga Khan University Medical College, Pakistan.
Pediatr Blood Cancer. 2015 Oct;62(10):1700-8. doi: 10.1002/pbc.25583. Epub 2015 May 15.
Acute lymphoblastic leukemia (ALL) is the most common cancer of childhood. Some evidence suggests differences in clinical and cytogenetic characteristics of ALL based on geographic and ethnic variations. However, data on ALL characteristics and early outcome of therapy from low/middle-income countries such as Pakistan are scanty.
A prospective, multi-institutional cohort study in Karachi enrolled 646 newly diagnosed children with ALL over 3 years. Standard forms were used to collect demographic, clinical, and laboratory data at presentation and at the end of induction.
Of the total, 66.1% (n = 427) were males. Median age was 6 (mean ± SE 6.87 ± 0.16; range 0.16-18) years. The most common clinical presentation was fever (88.7%). BPC-ALL was diagnosed in 78.5%, while 17.5% had T-ALL; 28.8% had a WBC >50 × 10(9) /L. With 316 patients karyotyped, hypodiploidy and hyperdiploidy were seen in 5.1% and 10.7%, respectively. Of those tested, ETV6-RUNX1 translocation was detected in 13.2%, while BCR-ABL1 translocation and MLL gene rearrangements were seen in 7.3% and 4.6%, respectively. The cumulative loss to follow up before and during induction was 12.8% (n = 83) and 11.5% (n = 74) died before or during this phase. Induction was successfully completed by only 75.6% (n = 489) of the entire cohort and 69.6% (n = 450) achieved remission.
These patients had ALL with higher risk features than that reported from developed countries. One quarter failed to complete induction chemotherapy. This suboptimal result requires further study and development of innovative interventions, particularly focusing on the causes and solutions for late referral, abandonment, and infections.
急性淋巴细胞白血病（ALL）是儿童期最常见的癌症。一些证据表明，ALL的临床和细胞遗传学特征因地理和种族差异而有所不同。然而，来自巴基斯坦等低收入/中等收入国家的ALL特征和早期治疗结果的数据很少。
在卡拉奇进行的一项前瞻性、多机构队列研究，在3年期间招募了646名新诊断的ALL儿童。使用标准表格在就诊时和诱导治疗结束时收集人口统计学、临床和实验室数据。
在总数中，66.1%（n = 427）为男性。中位年龄为6岁（平均±标准误6.87 ± 0.16；范围0.16 - 18岁）。最常见的临床表现是发热（88.7%）。78.5%诊断为BPC-ALL，而17.5%为T-ALL；28.8%的白细胞计数>50×10⁹/L。在316例进行核型分析的患者中，分别有5.1%和10.7%出现亚二倍体和超二倍体。在检测的患者中，13.2%检测到ETV6-RUNX1易位，而BCR-ABL1易位和MLL基因重排分别见于7.3%和4.6%。诱导治疗前和诱导治疗期间的累计失访率为12.8%（n = 83），在此阶段之前或期间有11.5%（n = 74）死亡。整个队列中只有75.6%（n = 489）成功完成诱导治疗，69.6%（n = 450）实现缓解。
这些患者的ALL具有比发达国家报告的更高风险特征。四分之一的患者未能完成诱导化疗。这一不理想的结果需要进一步研究并开发创新干预措施，尤其要关注延迟转诊、放弃治疗和感染的原因及解决方案。
