Department of Pediatric and Adolescent Medicine, Aarhus University Hospital, Aarhus, Denmark.
Department of Pediatric and Adolescent Medicine, Aalborg University Hospital, Aalborg, Denmark.
PLoS One. 2020 Aug 11;15(8):e0237530. doi: 10.1371/journal.pone.0237530. eCollection 2020.
Acute lymphoblastic leukemia (ALL) may present with arthritis implying the risk of being misdiagnosed as juvenile idiopathic arthritis (JIA). The aim of this study was to identify predictors for ALL based on clinical and laboratory information.
This cross-sectional, retrospective study compared clinical presentation and laboratory results of 26 children with ALL and arthritis versus 485 children with JIA (433 non-systemic, 52 systemic JIA). Using a Bayesian score approach the findings were evaluated by calculating odds ratios (OR) and lnOR as a measure of diagnostic weight.
Distinction on clinical grounds was difficult, as even a high number of joints involved did not exclude ALL. One or more hematologic cell counts were low (Hb <10 g/dL, platelet count <100 x 109/L, neutrophil count < 1.0 x 109/L) in 92% with ALL, 25% with systemic JIA and 10% with non-systemic JIA. Neutropenia and thrombocytopenia had the highest ORs of 128 (95% CI 43-387) and 129 (95% CI 26-638), each giving a diagnostic weight of 4. The estimated risks of ALL were 0.2% with normal cell counts and 9%, 67% and 100% when one, two or three cell lines were affected.
A simple count of cell lines with low counts can serve as a basic diagnostic strategy. Children with tri- or bilinear involvement should be referred to a bone marrow, and those with unilinear involvement a thorough screen for further evidence of ALL (organomegaly, ESR, LDH, uric acid, and blood smear).
急性淋巴细胞白血病（ALL）可表现为关节炎，这意味着存在被误诊为幼年特发性关节炎（JIA）的风险。本研究旨在根据临床和实验室信息确定 ALL 的预测因素。
这项横断面、回顾性研究比较了 26 例 ALL 伴关节炎患儿与 485 例 JIA 患儿（433 例非全身型 JIA，52 例全身型 JIA）的临床表现和实验室结果。使用贝叶斯评分方法，通过计算比值比（OR）和 lnOR（作为诊断权重的衡量标准）评估发现。
仅凭临床标准很难区分，因为即使累及关节数量多也不能排除 ALL。92%的 ALL 患儿存在一种或多种血细胞计数降低（Hb<10g/dL、血小板计数<100×109/L、中性粒细胞计数<1.0×109/L），25%的全身型 JIA 患儿和 10%的非全身型 JIA 患儿存在这种情况。中性粒细胞减少症和血小板减少症的 OR 最高，分别为 128（95%CI 43-387）和 129（95%CI 26-638），每项的诊断权重均为 4。细胞计数正常时 ALL 的估计风险为 0.2%，当一条、两条或三条细胞系受影响时，风险分别为 9%、67%和 100%。
简单计数细胞计数降低的细胞系可作为基本的诊断策略。三线或两线受累的患儿应转至骨髓，一线受累的患儿应进行全面筛查以进一步寻找 ALL 的证据（肝脾肿大、ESR、LDH、尿酸和血涂片）。
