McDonnell Alicia, Bride Karen L, Lim Derick, Paessler Michele, Witmer Char M, Lambert Michele P
Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
Division of Hematology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
Pediatr Blood Cancer. 2018 Feb;65(2). doi: 10.1002/pbc.26812. Epub 2017 Sep 17.
Differentiating childhood immune thrombocytopenia (ITP) from other cause of thrombocytopenia remains a diagnosis of exclusion. Additionally factors that predict bleeding risk for those patients with ITP are currently not well understood. Previous small studies have suggested that immature platelet fraction (IPF) may differentiate ITP from other causes of thrombocytopenia and in combination with other factors may predict bleeding risk.
We performed a retrospective chart review of thrombocytopenic patients with an IPF measured between November 1, 2013 and July 1, 2015. Patients were between 2 months and 21 years of age with a platelet count <50 × 10 /l. Each patient chart was reviewed for final diagnosis and bleeding symptoms. A bleeding severity score was retrospectively assigned.
Two hundred seventy two patients met inclusion criteria, 97 with ITP, 11 with bone marrow failure (BMF), 126 with malignancy, and 38 with other causes of thrombocytopenia. An IPF > 5.2% differentiated ITP from BMF with 93% sensitivity and 91% specificity. Absolute immature platelet number (AIPN) was significantly lower in ITP patients with severe to life-threatening hemorrhage than those without, despite similar platelet counts. On multivariate analysis, an IPF < 10.4% was confirmed as an independent predictor of bleeding risk at platelet counts <10 × 10 /l in patients with ITP.
IPF measurement alone has utility in both the diagnosis of ITP and identifying patients at increased risk of hemorrhage. Further study is required to understand the pathophysiological differences of ITP patients with lower IPF/AIPN.
鉴别儿童免疫性血小板减少症(ITP)与其他血小板减少原因仍然是一种排除性诊断。此外,目前对于ITP患者出血风险的预测因素尚不清楚。以往的小型研究表明,未成熟血小板比例(IPF)可能有助于鉴别ITP与其他血小板减少原因,并且与其他因素结合可能预测出血风险。
我们对2013年11月1日至2015年7月1日期间测量过IPF的血小板减少症患者进行了回顾性病历审查。患者年龄在2个月至21岁之间,血小板计数<50×10⁹/L。对每位患者的病历进行审查以确定最终诊断和出血症状。回顾性地指定了出血严重程度评分。
272名患者符合纳入标准,其中97例为ITP,11例为骨髓衰竭(BMF),126例为恶性肿瘤,38例为其他血小板减少原因。IPF>5.2%鉴别ITP与BMF的敏感性为93%,特异性为91%。尽管血小板计数相似,但有严重至危及生命出血的ITP患者的绝对未成熟血小板数量(AIPN)明显低于无出血的患者。多因素分析显示,IPF<10.4%被确认为ITP患者血小板计数<10×10⁹/L时出血风险的独立预测因素。
单独测量IPF在ITP诊断和识别出血风险增加的患者中均有作用。需要进一步研究以了解IPF/AIPN较低的ITP患者的病理生理差异。