摘要:目的：抗心磷脂抗体，β2-糖蛋白I(β2gpI)抗体或狼疮抗凝物(LA)统称为抗磷脂自身抗体(APA)，在免疫性血小板减少症(ITP)患者中检测到上述抗体，其与ITP之间的关系仍然是一个值得争论的问题。特别是，APA谱是否可能影响ITP的临床过程尚不清楚。

方法：在本研究中，我们报告了我们在治疗一组存在APA的ITP患者中的经验，关注不同抗磷脂抗体的临床结果和对治疗的反应。

结果：满足ITP标准的159例患者中有37例(23.2%)血小板计数≤50×10^9 / L，且在ITP发病时APA检测呈阳性。23(62.1%)名患者接受了至少一种ITP治疗。与其他APA患者相比，APA三重阳性的14名受试者(37.8%)表现为血小板中位数显着降低(p =0 .006)。在这些具有三重阳性的ITP受试者中，85.7%由于低血小板计数而需要治疗，而相比之下存在非三重阳性APA的ITP患者仅47.8%(p =0 .0094)需要治疗。接受免疫抑制剂治疗的ITP / APA患者血栓形成率较高(p = 0.024)，同样接受类固醇治疗的患者的血栓形成发生率也明显升高(p <0.001)。在考虑治疗时，与非三重阳性患者相比，ITP /三阳性患者的CR和SR率显着更高(p =0 .021和p = 0.005)。

结论：APA的存在可能会影响ITP患者的预后。

附原文：

Abstract OBJECTIVES: The relevance of detecting antibodies against anticardiolipin, β2-glycoprotein I (β2gpI) or lupus anticoagulant (LA), collectively called antiphospholipid autoantibodies (APA), in subjects with immune thrombocytopenia (ITP) is still a debated issue. In particular, whether APA profile may affect the clinical course of ITP is unknown.

METHODS: In this study, we report

our experience in a cohort of ITP patients with APA with specific interest to the relevance of different antiphospholipid antibody profiles in clinical outcome and response to treatment.

RESULTS: Thirty-seven out of 159 patients (23.2%) fulfilling ITP criteria had a platelet count ≤50 × 109/L and tested positive at APA at ITP onset. Twenty-three (62.1%) patients received at least one line of treatment for ITP. Fourteen subjects (37.8%) showing triple positivity for APA showed a significantly lower median platelet count compared to other APA patients (p = .006). Among these ITP subjects with triple positivity, 85.7% needed a treatment because of low platelet count compared to 47.8% ITP patients with non-triple-positive APA (p = .0094). ITP/APA subjects who received immunosuppressors had a higher rate of thrombosis (p = .024) as well as thrombosis developed in subjects who were on steroid therapy at a significantly higher dosage than subjects who did not develop thrombotic episodes (p < .001). When considering treatment, CR and SR rate were significantly higher in ITP/triple-positive patients compared to non-triple-positive subjects (p = .021 and p = .005).

CONCLUSIONS: The profile of APA may affect the outcome of patients with ITP.

引自：Frison L1, Lombardi A1, Caputo I1, et al, Relevance of antiphospholipid antibody profile in the clinical outcome of ITP: a single-centre study.Hematology. 2019 Dec;24(1):134-138. doi: 10.1080/10245332.2018.1532649. Epub 2018 Oct 25.