骨髓增殖性疾病四例并文献复习

 目的　总结分析4例典型骨髓增殖性疾病（MPD）患者的诊治经过，并进行相关文献复习。方法　介绍1例真性红细胞增多症、2例原发性血小板增多症和1例原发性骨髓纤维化患者的诊治经过。结果　4例患者分别采用干扰素、干扰素联合羟基脲、同胞HLA相合异基因造血干细胞移植治疗，均获得满意疗效。结论　JAK2 V617F基因突变的检测使MPD的临床诊断变得更容易；真性红细胞增多症和原发性血小板增多症患者的治疗重点为预防并发症，而对于部分高危原发性骨髓纤维化患者应考虑异基因造血干细胞移植。

Diagnosis and treatment of four patients with myeloproliferative neoplasms and a literature review

Objective To analyze the clinical practice of the diagnosis and treatment of four patients with myeloproliferative diseases (MPD) and review the relative literatures. Methods The progress of diagnosing and treating one polycythemia vera patient, two essential thrombocythemia patients and one primary myelofibrosis patient were introduced. Results Interferon alone, interferon combined with hydroxyurea and HLA matched sible allogeneic hematopoietic stem cell transplantation were carried out separately, the satisfying outcomes were shown. Conclusion Detection of genetic mutation of JAK2 V617F has markedly facilitated the approach to clinical diagnosis of MPD. The therapeutic aim for patients with polycythemia vera and essential thrombocythemia is preventing the occurrence thromboembolic events, while for some high-risk primary myelofibrosis patient, allogeneic stem cell transplantation should be considered.