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抗磷脂综合征高危患者的血小板减少症。

Thrombocytopenia in high-risk patients with antiphospholipid syndrome.

机构信息

Department of Cardiac Thoracic and Vascular Sciences, Cardiology Clinic, Thrombosis Center, University of Padova, Padova, Italy.

Department of Medicine, Clinical Rheumatology, University of Padova, Padova, Italy.

出版信息

J Thromb Haemost. 2018 Mar;16(3):529-532. doi: 10.1111/jth.13947. Epub 2018 Jan 25.

Abstract

UNLABELLED

Essentials The prevalence of thrombocytopenia in patients with antiphospholipid syndrome is not well defined. We studied triple positive patients with antiphospholipid syndrome and its catastrophic variant. Prevalence of thrombocytopenia was 6% and 100% in patients who developed the catastrophic form. In triple positive patients thrombocytopenia is low and platelets drop during the catastrophic form.

SUMMARY

Background Thrombocytopenia is the most common non-criteria hematological feature in patients with antiphospholipid syndrome (APS). This condition is more common in patients with catastrophic APS (CAPS). Objectives To evaluate the prevalence of thrombocytopenia in a large series of high-risk patients with APS, and to assess the behavior of the platelet count during CAPS. Methods/Patients This was a cross-sectional study in which we analyzed the platelet counts of a homogeneous group of high-risk APS patients (triple-positive). Six of these patients developed a catastrophic phase of the disease, and the platelet count was recorded before the acute phase, during the acute phase, and at recovery. Results The mean platelet count in 119 high-risk triple-positive patients was 210 × 10 L . With a cut-off value for thrombocytopenia of 100 × 10 L , the prevalence of thrombocytopenia was 6% (seven patients). No difference between primary APS and secondary APS was found. In patients who suffered from CAPS, a significant decrease from the basal count (212 ± 51 × 10 L ) to that at the time of diagnosis (60 ± 33 × 10 L ) was observed. The platelet count became normal again at the time of complete remission (220 ± 57 × 10 L ). A decrease in platelet count always preceded the full clinical picture. Conclusions This study shows that, in high-risk APS patients, the prevalence of thrombocytopenia is low. A decrease in platelet count was observed in all of the patients who developed the catastrophic form of the disease. A decrease in platelet count in high-risk APS patients should be considered a warning signal for disease progression to CAPS.

摘要

背景

血小板减少症是抗磷脂综合征(APS)患者最常见的非标准血液学特征。这种情况在灾难性 APS(CAPS)患者中更为常见。目的:评估大量高危 APS 患者中血小板减少症的患病率,并评估血小板计数在 CAPS 期间的变化情况。方法/患者:这是一项横断面研究,我们分析了一组同质的高危 APS 患者(三联阳性)的血小板计数。其中 6 例患者发生了疾病的灾难性阶段,并记录了急性前期、急性期间和恢复期的血小板计数。结果:119 例高危三联阳性患者的平均血小板计数为 210×10^9/L。以血小板减少症的截断值为 100×10^9/L 计算,血小板减少症的患病率为 6%(7 例)。原发性 APS 和继发性 APS 之间没有差异。在发生 CAPS 的患者中,与基础计数(212±51×10^9/L)相比,诊断时的血小板计数显著下降(60±33×10^9/L)。在完全缓解时,血小板计数再次恢复正常(220±57×10^9/L)。血小板计数下降总是先于完全的临床症状出现。结论:本研究表明,在高危 APS 患者中,血小板减少症的患病率较低。所有发生灾难性疾病形式的患者均观察到血小板计数下降。高危 APS 患者的血小板计数下降应被视为疾病进展为 CAPS 的警告信号。

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