University Hospitals Cleveland Medical Center and Case Western Reserve University School of Medicine, Cleveland, OH, United States of America.
University Hospitals Cleveland Medical Center and Case Western Reserve University School of Medicine, Cleveland, OH, United States of America.
Thromb Res. 2022 Jul;215:30-36. doi: 10.1016/j.thromres.2022.05.008. Epub 2022 May 20.
Thrombotic thrombocytopenic purpura (TTP) demands rapid initiation of therapeutic plasma exchange to avoid severe complications associated with it. ADAMTS13 activity <10% defines TTP, however, this is a send-out test at most institutions. We have previously reported our experience looking at absolute immature platelet counts (A-IPC) in TTP patients. Thus, we compared A-IPC on admission to the PLASMIC score to predict ADAMTS13 deficiency.
Of seventy-two patients identified, 52 met inclusion criteria. All patients were suspected of new-onset TTP and had A-IPC on admission. Of these patients, 25/52 were later shown to have ADAMTS13 <10%, defined as TTP group, and 27/52 had ADAMTS13 >10% and are henceforth classified as non-TTP.
Patients with TTP were found to have A-IPC below reference range (<1.5 × 10/L) and responded to therapy as shown by fold-increases in A-IPC (p < 0.0001), neither seen in non-TTP patients. A-IPC had a significant correlation with ADAMTS13 deficiency (p = 0.0001) with high sensitivity, specificity, positive and negative predictive values. Comparison of A-IPC to PLASMIC score indicated that the former identified all TTP patients compared to PLASMIC score even in patients obtaining scores of 4 and 5. Finally, Receiver Operating Characteristic curves showed A-IPC had area under the curve of 0.986.
A-IPC below reference range and A-IPC fold-increases were only observed in TTP patients. There was strong association between A-IPC and ADAMTS13 deficiency and A-IPC predicted patients with ADAMTS13 deficiency. Future larger studies are needed to determine ways to apply findings in suspected TTP patients.
血栓性血小板减少性紫癜(TTP)需要迅速开始治疗性血浆置换以避免其相关的严重并发症。ADAMTS13 活性<10%定义为 TTP,但这是大多数机构的外送检测。我们之前曾报告过我们在 TTP 患者中观察绝对未成熟血小板计数(A-IPC)的经验。因此,我们比较了入院时的 A-IPC 与 PLASMIC 评分,以预测 ADAMTS13 缺乏。
在确定的 72 名患者中,有 52 名符合纳入标准。所有患者均怀疑为新发 TTP,且入院时均有 A-IPC。这些患者中,25/52 例随后被证实 ADAMTS13<10%,定义为 TTP 组,27/52 例 ADAMTS13>10%,此后归类为非 TTP。
TTP 患者的 A-IPC 低于参考范围(<1.5×10/L),且 A-IPC 的倍数增加表明对治疗有反应(p<0.0001),而非 TTP 患者则没有。A-IPC 与 ADAMTS13 缺乏具有显著相关性(p=0.0001),具有高灵敏度、特异性、阳性和阴性预测值。与 PLASMIC 评分相比,A-IPC 可识别所有 TTP 患者,而 PLASMIC 评分即使在获得 4 分和 5 分的患者中也可以。最后,接受者操作特征曲线显示 A-IPC 的曲线下面积为 0.986。
只有 TTP 患者观察到低于参考范围的 A-IPC 和 A-IPC 倍数增加。A-IPC 与 ADAMTS13 缺乏之间存在很强的关联,A-IPC 可预测 ADAMTS13 缺乏的患者。未来需要更大的研究来确定如何将这些发现应用于疑似 TTP 患者。