A Retrospective Study Of Prognostic Value Of Splenomegaly In Patients With Primary Myelofibrosis Clinical Features And Survival Analysis In Post-polycythemia Vera And Post-essential Thrombocytosis Myelofibrosis Effection Analysis Of Low-dose Thalidomide A

ObjectiveTo investigate the prognostic value of splenomegaly in patients with primary myelofibrosis (PMF).MethodsEight hundred and seventy-four PMF patients were retrospectively analyzed. Kaplan-Meier method, Log-rank test and COX regression model were used to evaluate clinic factors that influence the prognosis.ResultsIn our cohort,495(56.6%) cases were with palpable spleen. There were significant differ ences in the distribution of hemoglobin, white blood cell count, platelet count, circulating blasts, age>65years old, transfusion dependence, JAK2V617F mutation and dynamic international prognostic scoring system(DIPSS) risk group(P<0.05) between the cases with palpable spleen and without palpable spleen. The overall survival (OS) of patients with low risk, intermediate risk and high risk according to the modified international prognostic scoring system (IPSS) for Chinese were288,64and29months, respectively(P<0.05). Using the modified DIPSS for Chinese, the OS were288,61and28months(P<0.05). Splenomegaly had independent prognostic significance in univariate analysis and multivariate analysis (P=0.000,0.009).ConclusionSplenomegaly indicated favorable prognostic effect on PMF patients independent of the DIPSS categories. ObjectiveTo investigate the risk factors of evolution to post-polycythemia vera and post-essential thrombocythemia myelofibrosis(Post-PV/ET MF) in patients with polycythemia vera(PV) or essential thrombocytosis(ET),and the prognostic factors of Post-PV/ET MF.Methods2533myeloproliferative neoplasm (MPN) patients (695with PV,964with ET, and874with primary myelofibrosis) were retrospectively analyzed. Kaplan-Meier method, Log-rank test and COX regression model were used to evaluate clinic factors that influence the prognosis and the evolution to Post-PV/ET MF.ResultsAmong PV patients, the median time of myelofibrosis transformation in the patients, whose white blood cell(WBC) count were more than15109/L, was196months versus not reached in the group of WBC<15109/L (P<001). Among ET patients, in the group of platelet count(PLT)≤600109/L, the median time of myelofibrosis transformation was132months versus238months in the group of PLT>600109/L (P=0.011). By Cox regression analysis, the independent predictors of death were hemoglobin(HB)<100g/L (hazard ratio[HR]=5.967,95%confidence interval [CI],1.031-34.520;P=0.046) and PLT<100109/L (HR=15.273,95%CI,1.735-134.285;P=0.014) and risk categorization is accordingly defined:low risk(0adverse factor) and high risk (≥1adverse factors).The corresponding median survivals were152and47months (P=0.037).ConclusionThe current study indicated that WBC>15109/L was strongly associated with myelofibrotic transformation in PV patients and PLT<600109/L in ET patients. HB <100g/L and PLT<100109/L had independent prognostic significance in Post-PV/ET MF patients. Objective To observe the clinical effects of low-dose thalidomide (THAL) and prednisone (PRED) with or without danazol (DANA) in patients with primary myelofibrosis (PMF) associated anemia.MethodsA retrospective study of fifty-eight PMF patients with anemia (Hb<100g/L) were included. A cohort of28patients were treated with THAL and PRED (THAL-PRED group), and another of30patients with THAL, PRED and DANA (THAL-PRED-DANA group). The hematological response was assessed according to the modified criteria of the International Working Group in2006, and the myelofibrosis degree was evaluate at3and12month after receiving treatment.ResultsThe total response rate was56.9%(33/58) including1.7%(1/58) partial remission (PR) and55.2%(32/58) clinical improvement(CI). There were no statistical difference in the response rate between THAL-PRED and THAL-PRED-DANA groups (50.0%vs63.3%, P=0.306).However, the median response duration time of clinical improvement, erythroid response (CI-E) and total response prolonged in THAL-PRED-DANA than THAL-PRED group (61.5w vs22w, P=0.015;75w vs30w, P=0.007, respectively). Myelofibrosis degree at3and12months after treatment decreased significantly than before treatment (P=0.000and0.005).Side-effects in both groups were only grade1-2.ConclusionsLow-dose THAL together with PRED appears to be effective in the treatment of PMF-associated anemia, and the response duration time will prolong significantly if combined with DANA. ObjectiveTo investigate the value of myeloproliferative neoplasms Symptom Assessment Form (MPN-SAF) and MPN-SAF total symptom score (MPN-SAF-TSS) in assessing the quality of life in myeloproliferative neoplasms (MPNs) patients.MethodsA cohort of628MPN patients were administered in171patients with polycythemia vera (PV),269patients with essential thrombocytosis (ET),188patients with myelofibrosis (MF). MPN-SAF and MPN-SAF-TSS load analysis cases and incidence of symptoms in patients with MPN, and by the European Organisation for Research and Treatment of Cancer Quality of Life Questionnaire-C30(EORTC-QLQ-C30) to verify its reliability and validity.ResultsFatigue was the most common symptom (80.3%prevalence), and the highest average severity of all the symptoms was low libido/sexual dysfunction (mean score of4.59). Disease burden was greatest in patients with MF followed by PV and ET patients except headache and itching. Using the MPN-SAF-TSS analysis, we found MF patients show highest burden of symptoms (mean score of28.7), followed by the PV patients (mean score of19.3), and finally the ET patients (mean score of16.9). MPN-SAF and MPN-SAF TSS showed strong correlations with the EORTC-QLQ-C30in functional areas (physical, role, cognitive, emotional and social fields) and symptoms areas.ConclusionBoth MPN-SAF and MPN-SAF TSS are reliable instrument that is available in the evaluation of MPN subtypes.