Hashkes Philip J, Wright Bridget M, Lauer Michael S, Worley Sarah E, Tang Anne S, Roettcher Philip A, Bowyer Suzanne L
Section of Pediatric Rheumatology, Cleveland Clinic, Cleveland, OH, USA.
J Pediatr Hematol Oncol. 2011 Aug;33(6):424-8. doi: 10.1097/MPH.0b013e31820998c4.
Approximately 30% of pediatric acute lymphoblastic leukemia patients present with musculoskeletal symptoms and are often referred first to a pediatric rheumatologist. We examined the survival and causes of death of these patients presenting to a pediatric rheumatologist and compared the rates with that reported in the hematology-oncology literature.
We used the Pediatric Rheumatology Disease Registry, including 49,023 patients from 62 centers, newly diagnosed between 1992 and 2001. Identifiers were matched with the Social Security Death Index censored for March 2005. Deaths were confirmed by death certificates, referring physicians, and medical records. Causes of death were derived by chart review or from the death certificate.
There were 7 deaths of 89 patients (7.9%, 95% confidence interval: 3.9%-15.4%) with acute lymphoblastic leukemia with a 5-year survival rate of 95.5% (88.3 to 98.3) and 10-year survival rate of 89.8% (79.0% to 95.2%). The causes of death were sepsis (bacterial and/or fungal) in 4 (57%) patients, the disease in 2 (29%) and post bone-marrow transplantation in 1 (14%).
The overall survival of patients with acute lymphoblastic leukemia seen first by pediatric rheumatologists is higher than the range reported in the pediatric oncology literature for the same period of diagnosis.
约30%的小儿急性淋巴细胞白血病患者表现出肌肉骨骼症状，通常首先被转诊至小儿风湿病专科医生处。我们研究了这些转诊至小儿风湿病专科医生处的患者的生存率和死亡原因，并将其发生率与血液学 - 肿瘤学文献中报道的进行比较。
我们使用了小儿风湿病疾病登记处的数据，其中包括1992年至2001年间新诊断的来自62个中心的49,023名患者。标识符与截至2005年3月审查的社会安全死亡指数进行匹配。通过死亡证明、转诊医生和病历确认死亡情况。死亡原因通过病历审查或死亡证明得出。
89例急性淋巴细胞白血病患者中有7例死亡（7.9%，95%置信区间：3.9% - 15.4%），5年生存率为95.5%（88.3%至98.3%），10年生存率为89.8%（79.0%至95.2%）。死亡原因包括4例（57%）患者发生败血症（细菌和/或真菌），2例（29%）患者因疾病本身，1例（14%）患者死于骨髓移植后。
小儿风湿病专科医生首先诊治的急性淋巴细胞白血病患者的总体生存率高于同期小儿肿瘤学文献报道的范围。
