疾病初发时急性淋巴细胞白血病与全身型幼年特发性关节炎的鉴别。

Discrimination of acute lymphoblastic leukemia from systemic-onset juvenile idiopathic arthritis at disease onset.

机构信息

Pediatric Rheumatology Unit, Faculdade de Medicina, Instituto da Criança, Universidade de São Paulo, São Paulo, Brazil.

出版信息

Clinics (Sao Paulo). 2011;66(10):1665-9. doi: 10.1590/s1807-59322011001000001.

DOI:10.1590/s1807-59322011001000001

PMID:22012035

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3180165/

Abstract

OBJECTIVE

To assess clinical and laboratory features that differentiate acute lymphoblastic leukemia from systemic juvenile idiopathic arthritis at disease onset.

METHODS

Fifty-seven leukemia patients with musculoskeletal involvement, without blasts on peripheral blood and without glucocorticoid therapy at disease onset and 102 systemic juvenile idiopathic arthritis patients (International League of Associations for Rheumatology criteria) were retrospectively evaluated. The following features were examined: fever, rheumatoid rash, arthritis, limb pain, hepatomegaly, splenomegaly, pericarditis, myocarditis, pleuritis, weight loss, bleeding, anemia, leukopenia, neutropenia, thrombocytopenia, erythrocyte sedimentation rate, and lactic dehydrogenase levels.

RESULTS

The median age at disease onset was significantly higher in leukemia patients than in those with systemic-onset juvenile idiopathic arthritis (5.8 vs. 3.8 years). In addition, the frequencies of limb pain, hepatomegaly, weight loss and hemorrhagic manifestations were significantly higher in leukemia patients than in systemic-onset juvenile idiopathic arthritis patients (70% vs. 1%, 54% vs. 32%, 30% vs. 8%, and 9% vs. 0%, respectively). Likewise, the frequencies of anemia, leukopenia, neutropenia, thrombocytopenia and high lactic dehydrogenase levels were statistically higher in leukemia patients than in patients with systemic-onset juvenile idiopathic arthritis (88% vs. 57%, 39% vs. 1%, 60% vs. 1%, 77% vs. 1%, and 56% vs. 14%, respectively). Remarkably, multivariate analysis revealed that limb pain (OR = 553; 95% CI = 46.48-6580.42) and thrombocytopenia (OR = 754.13; 95% CI = 64.57-8806.72) were significant independent variables that differentiated leukemia from systemic-onset juvenile idiopathic arthritis. The R2 of the Nagelkerke test was 0.91, and the Kaplan-Meier survival curves were similar for acute lymphoblastic leukemia patients with and without limb pain.

CONCLUSION

Our study emphasizes the importance of investigating leukemia in patients presenting with musculoskeletal manifestations and, in particular, limb pain associated with thrombocytopenia.

摘要

目的

评估在疾病发作时区分急性淋巴细胞白血病和全身型幼年特发性关节炎的临床和实验室特征。

方法

回顾性评估了 57 例伴有肌肉骨骼受累、外周血无blasts、疾病发作时无糖皮质激素治疗的白血病患者和 102 例全身型幼年特发性关节炎患者（国际风湿病联盟协会标准）。检查了以下特征：发热、类风湿性皮疹、关节炎、肢体疼痛、肝肿大、脾肿大、心包炎、心肌炎、胸膜炎、体重减轻、出血、贫血、白细胞减少、中性粒细胞减少、血小板减少、红细胞沉降率和乳酸脱氢酶水平。

结果

白血病患者的中位发病年龄显著高于全身型幼年特发性关节炎患者（5.8 岁 vs. 3.8 岁）。此外，肢体疼痛、肝肿大、体重减轻和出血表现的发生率在白血病患者中显著高于全身型幼年特发性关节炎患者（70% vs. 1%、54% vs. 32%、30% vs. 8%和 9% vs. 0%）。同样，贫血、白细胞减少、中性粒细胞减少、血小板减少和高乳酸脱氢酶水平的发生率在白血病患者中也显著高于全身型幼年特发性关节炎患者（88% vs. 57%、39% vs. 1%、60% vs. 1%、77% vs. 1%和 56% vs. 14%）。值得注意的是，多变量分析显示，肢体疼痛（OR = 553；95%CI = 46.48-6580.42）和血小板减少（OR = 754.13；95%CI = 64.57-8806.72）是区分白血病和全身型幼年特发性关节炎的显著独立变量。Nagelkerke 检验的 R2 为 0.91，有肢体疼痛和无肢体疼痛的急性淋巴细胞白血病患者的 Kaplan-Meier 生存曲线相似。