Sinigaglia Riccardo, Gigante Cosimo, Bisinella Gianluca, Varotto Stefania, Zanesco Luigi, Turra Sisto
Sandro Agostini Spine Surgery Unit, Padua University Hospital, Padua, Italy.
J Pediatr Orthop. 2008 Jan-Feb;28(1):20-8. doi: 10.1097/BPO.0b13e31815ff350.
In children, acute leukemia (AL) at presentation can mimic several orthopaedic pathologies, so that a variable delay of the correct diagnosis is often reported.
To define more clearly the clinical and radiological musculoskeletal manifestations of leukemia in children, 122 affected children referred from 1984 to 1999 to our Pediatric Onco-Hematologic Clinic were retrospectively reviewed. Average age at diagnosis was 6.6 years (from 7 months to 17 years). Seventy-three (60%) were boys and adolescent boys, 49 (40%) were girls and adolescent girls. One hundred two (83.6%) had acute lymphoblastic leukemia, 20 (16.4%) had acute myeloid leukemia. The mean follow-up was 8.2 years for the 104 survivors and 2.5 years for the 18 nonsurvivors. The chi2 test was used to perform the statistical analyses.
At presentation, complaints related to the musculoskeletal system were frequent (38.3%), including pain (34.4%), functional impairment (22.9%), limping (12.3%), swelling (10.6%), and joint effusion (5.7%). At presentation, 40.2% of children had at least 1 radiographic abnormality. In order, they were osteolysis (13.1%), metaphyseal bands (9.8%), osteopenia (9%), osteosclerosis (7.4%), permeative pattern (5.7%), pathological fractures (5.7%), periosteal reactions (4.1%), and mixed lysis-sclerosis lesions (2.5%). Different from previous reports, late radiographic lesions were uncommon (5.7%), probably because of milder newer medication protocols. They included avascular necrosis (3.3%), vertebral collapses (1.6%), and osteolysis (0.8%).
Both clinical and radiological changes had various and no uniform localization. Poor correlation was found between symptoms and radiological lesions. Survival rates in children with AL were 95.8% at 1 year, 89.6% at 3 years, 85.8% at 5 years, and 83.4% at 10 and at 13 years. Radiographic abnormalities (P = 0.400), type of leukemia (P = 0.291), sex (P = 0.245), and white blood cell count at presentation (P = 0.877) were not prognostic factors. The presence of multiple bone lesions did not affect the survival rate (P=0.632). As early diagnosis significantly decreases morbidity and mortality of AL, the orthopaedist should suspect AL in any child with unexplained persistent skeletal pain or radiographic alterations. Accurate history, general physical examination, and complete blood cell count tests should address the suspicion, which is confirmed by a peripheral and/or iliac crest bone marrow biopsy.
儿童急性白血病（AL）在初诊时可能类似多种骨科疾病，因此常出现正确诊断的延迟。
为更清楚地界定儿童白血病的临床和放射学肌肉骨骼表现，对1984年至1999年转诊至我们儿科肿瘤血液科的122例患病儿童进行回顾性分析。诊断时的平均年龄为6.6岁（7个月至17岁）。73例（60%）为男孩和青少年男性，49例（40%）为女孩和青少年女性。102例（83.6%）患有急性淋巴细胞白血病，20例（16.4%）患有急性髓细胞白血病。104例幸存者的平均随访时间为8.2年，18例非幸存者的平均随访时间为2.5年。采用卡方检验进行统计分析。
初诊时，与肌肉骨骼系统相关的主诉很常见（38.3%），包括疼痛（34.4%）、功能障碍（22.9%）、跛行（12.3%）、肿胀（10.6%）和关节积液（5.7%）。初诊时，40.2%的儿童至少有1项放射学异常。依次为骨质溶解（13.1%）、干骺端带（9.8%）、骨质减少（9%）、骨质硬化（7.4%）、浸润型（5.7%）、病理性骨折（5.7%）、骨膜反应（4.1%）和溶骨-硬化混合性病变（2.5%）。与既往报道不同，晚期放射学病变不常见（5.7%），可能是因为采用了较温和的新治疗方案。包括缺血性坏死（3.3%）、椎体塌陷（1.6%）和骨质溶解（0.8%）。
临床和放射学改变具有多样性且定位不统一。症状与放射学病变之间相关性较差。AL患儿1年生存率为95.8%，3年生存率为89.6%，5年生存率为85.8%，10年和13年生存率为83.4%。放射学异常（P = 0.400）、白血病类型（P = 0.291）、性别（P = 0.245）和初诊时白细胞计数（P = 0.877）均不是预后因素。多发骨病变的存在不影响生存率（P = 0.632）。由于早期诊断可显著降低AL的发病率和死亡率，骨科医生应对任何有不明原因持续性骨骼疼痛或放射学改变的儿童怀疑AL。准确的病史、全面的体格检查和全血细胞计数检查应有助于该怀疑，外周血和/或髂嵴骨髓活检可确诊。
