伴发于真性红细胞增多症的红斑性肢痛症1例报道及文献复习

Erythromelalgia accompanying polycythemia vera:A case report and review of the literature

目的研究伴发于真性红细胞增多症的红斑性肢痛症发病机制、临床表现、病理生理特点。方法分析1例确诊真性红细胞增多症患者的临床表现、实验室及辅助检查、尸检病理,血小板计数动态监测结果。结果该病例有骨髓增殖性疾病病史,后期间断出现肢体末端烧灼样疼痛、痛性红斑,直至干性坏疽;多系统多脏器血栓形成;同时伴有血小板计数的进行性减少。结论血栓形成是红斑性肢痛症各种临床表现的主要因素,应尽可能做到早期诊断,早期运用抗血小板药物治疗,发展至终末期治疗效果不佳。

Objective To study the mechanism,clinical manifestations and pathophysiological characteristics of erythromelalgia accompanying polycythemia vera.Methods Clinical manifestations,laboratory test and auxiliary examinations,autopsy report and platelet count of a patient with erythromelalgia were analyzed.Results The patient with a history of myeloproliferative disease developed extremity burning pain,painful erythema and even dry gangrene with formation of thrombosis in multiple systems and organs accompanying progressively decreased platelet count.Conclusion Formation of thrombosis is the major factor for the different clinical manifestations of erythromelalgia,which should be early diagnosed and treated with anti-platelet drugs.