Guo Yan, Xu Wenwei, Dong Lin, Huang Ning, Bi Kehong
Department of Hematology, Shandong Provincial Qianfoshan Hospital Affiliated to Shandong University, Jinan, Republic of China.
Medicine (Baltimore). 2017 Jul;96(28):e7464. doi: 10.1097/MD.0000000000007464.
This case report describes the progression of primary myelofibrosis (PMF) to polycythemia vera (PV), and discuss its potential mechanisms.
The patient was admitted because of abdominal discomfort and enlarged spleen for 19 months.
A case of PMF progressed to PV was retrospectively analyzed. There were 19 months between the diagnosis of PMF and PV. The JAK2 V617F mutation was positive before and after the diagnosis of PV; however, new chromosomal abnormalities were detected during the progression.
For treatment of PMF, the danazol, calcitriol, and thalidomide were given. Then, the use of thalidomide and calcitriol was stopped, and hydroxyurea was started. For treatment of PV, interferon treatment was given, whereas hydroxyurea was continued.
After 30 months of the progression (at the recent follow-up), this patient had no obvious symptoms or thrombosis.
PMF rarely progresses to PV, however, the progression will significantly improve the quality of life and prognosis.
本病例报告描述了原发性骨髓纤维化（PMF）进展为真性红细胞增多症（PV）的过程，并探讨了其潜在机制。
患者因腹部不适和脾脏肿大19个月入院。
回顾性分析1例PMF进展为PV的病例。PMF诊断与PV诊断间隔19个月。PV诊断前后JAK2 V617F突变均为阳性；然而，进展过程中检测到新的染色体异常。
治疗PMF时，给予达那唑、骨化三醇和沙利度胺。之后，停用沙利度胺和骨化三醇，开始使用羟基脲。治疗PV时，给予干扰素治疗，同时继续使用羟基脲。
进展30个月后（最近一次随访时），该患者无明显症状或血栓形成。
PMF很少进展为PV，然而，这种进展将显著改善生活质量和预后。
