Gameiro Rita de Sousa, Rodrigues Ana, Gonçalves Fernando Martos, Graça José Pimenta da
Medicina Interna, Hospital Beatriz Ângelo, Loures, Portugal.
BMJ Case Rep. 2017 Mar 9;2017:bcr2016218851. doi: 10.1136/bcr-2016-218851.
Polycythaemia vera (PV) is the most common myeloproliferative neoplasm, characterised by increased red cell mass that can present as an unspecified symptom or a thrombohaemorrhagic event. Its diagnosis is based on the presence of erythrocytosis, the identification of the Janus kinase 2 mutation and bone marrow aspirate or biopsy alterations. The challenge of this disease lies on the treatment approach. Its cornerstone is phlebotomy, but depending on the vascular risk, it can include cytoreductive agents, low-dose aspirin or even anticoagulation. We present the case of a 75-year-old woman, whose inaugural presentation of PV was an arterial peripheral occlusion followed by three recurrent events in the same arterial region and a pulmonary embolism. A phlebotomy was initially performed and, after the diagnosis was made, the patient was initiated on low-dose aspirin and anticoagulation with favourable outcome.
真性红细胞增多症（PV）是最常见的骨髓增殖性肿瘤，其特征是红细胞量增加，可表现为非特异性症状或血栓出血事件。其诊断基于红细胞增多症的存在、Janus激酶2突变的鉴定以及骨髓穿刺或活检改变。这种疾病的挑战在于治疗方法。其基石是放血疗法，但根据血管风险，治疗可包括细胞减灭剂、低剂量阿司匹林甚至抗凝治疗。我们报告一例75岁女性病例，其PV的首发表现为动脉外周闭塞，随后在同一动脉区域出现三次复发事件及一次肺栓塞。最初进行了放血治疗，确诊后，患者开始接受低剂量阿司匹林和抗凝治疗，结果良好。
