Analysis of Clinical Features and Prognosis in 31 Patients with Primary Myelofibrosis / 中国实验血液学杂志

<p><b>OBJECTIVE</b>To investigate the clinical and laboratorial features of primary myelofibrosis (PMF) patients treated in our hospital, to analyze the risk factors influancing survival, and to compare several prognostic scoring systems.</p><p><b>METHODS</b>parameters about clinical and laboratorial features were taken from medical documents at diagnosis, univariate analysis was conducted by Kaplan-Meier method, the survival data were compared with Log-rank test, and a COX model was used for multivariate analyses.</p><p><b>RESULTS</b>In our center the anemia and JAK2V617F mutation were more common, while the abnormal karyotype was less common, the constitutional symptoms, splenomegaly, Hb level < 100 g/L and LDH are adverse factors for survival in univariate analysis. Constitutional symptoms and Plt count < 100 × 10(9)/L are adverse factors of survival according to multivariate analysis.</p><p><b>CONCLUSION</b>The anemia is more frequent in Chinese patients. Constitutional symptoms and Plt count < 100 × 10(9)/L are independent risk factors for survival. LILLI, modified IPSS, modified DIPSS are suitable to our data.</p>

Research progress of myeloproliferative neoplasms / 中国实验血液学杂志

The classic BCR-ABL negative myeloproliferative neoplasm (MPN) comprise polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF). These three disorders are characterized by stem cell-derived clonal myeloproliferation and presence of somatic mutations. The WHO diagnostic criteria for the classic BCR-ABL negative MPN has been revised in the 2008 edition by incorporating new information about their molecular pathogenesis. Robust prognostic system for PMF has already done, and those for PV and ET are under discussion. Treatment with novel drugs is promising, and allo-stem cell transplantation (allo-ASCT) is the only curative treatment for myelofibrosis, however, the patient selection and management before transplant have been discussed for a long time.