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伴有费城染色体阴性骨髓增殖性疾病的患者肝素诱导的血小板减少症和不常见的内脏静脉或脑静脉血栓形成。

Heparin-induced thrombocytopenia in patients with Philadelphia-negative myeloproliferative disorders and unusual splanchnic or cerebral vein thrombosis.

机构信息

Department of Medical and Surgical Sciences, University of Padua Medical School, Padua, Italy.

出版信息

Acta Haematol. 2010;123(3):140-5. doi: 10.1159/000280466. Epub 2010 Feb 4.

Abstract

BACKGROUND

Philadelphia-negative myeloproliferative disorders (Ph-MPD) are common causes of unusual splanchnic or cerebral vein thrombosis, which is treated with unfractionated heparin (UFH) or low-molecular-weight heparin (LMWH). Heparin-induced thrombocytopenia (HIT) is a dangerous potential complication of this therapy, but it has rarely been reported in Ph-MPD.

PATIENTS AND METHODS

We retrospectively reviewed clinical records of 29 patients with Ph-MPD who have been treated with UFH or LMWH for unusual splanchnic or cerebral vein thrombosis (3 cerebral sinus, 6 portal and 20 hepatic vein). The goal of the study was to determine the occurrence of new thrombotic events during heparin therapy secondary to HIT (HITT).

RESULTS

During heparin therapy, 5 out of the 29 patients (17%) developed a new thrombotic episode (pulmonary embolism) with a high clinical probability of HIT based on the 4 T's score even though not all the patients developed 'true' thrombocytopenia. A diagnosis of HIT was established in 2 patients (6.8%) through the presence of heparin-related antibodies.

CONCLUSIONS

Ph-MPD patients on heparin warrant careful monitoring and HIT has to be suspected whenever platelet counts drop or a new thrombosis is detectable.

摘要

背景

费城阴性骨髓增殖性疾病(Ph-MPD)是引起罕见内脏或脑静脉血栓形成的常见原因,常采用普通肝素(UFH)或低分子肝素(LMWH)进行治疗。肝素诱导的血小板减少症(HIT)是这种治疗方法的一种危险潜在并发症,但在 Ph-MPD 中很少有报道。

患者和方法

我们回顾性分析了 29 例 Ph-MPD 患者的临床记录,这些患者因罕见的内脏或脑静脉血栓形成(3 例脑窦、6 例门静脉和 20 例肝静脉)接受了 UFH 或 LMWH 治疗。研究的目的是确定因 HIT(HITT)继发于肝素治疗的新血栓形成事件的发生。

结果

在肝素治疗期间,29 例患者中有 5 例(17%)出现新的血栓形成事件(肺栓塞),根据 4T's 评分,HIT 的临床可能性很高,尽管并非所有患者都出现“真正”的血小板减少症。通过存在肝素相关抗体,在 2 例患者(6.8%)中确立了 HIT 的诊断。

结论

接受肝素治疗的 Ph-MPD 患者需要密切监测,并且每当血小板计数下降或出现新的血栓形成时,都应怀疑 HIT。

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