Department of Medical and Surgical Sciences, University of Padua Medical School, Padua, Italy.
Acta Haematol. 2010;123(3):140-5. doi: 10.1159/000280466. Epub 2010 Feb 4.
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.
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).
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.
Ph-MPD patients on heparin warrant careful monitoring and HIT has to be suspected whenever platelet counts drop or a new thrombosis is detectable.
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。
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。
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。
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。