A study of clinical characteristics and prognosis of primary myelofibrosis patients with thrombocytopenia in varied degrees
10.3760/cma.j.issn.0253-2727.2019.01.003
- VernacularTitle: 不同血小板水平原发性骨髓纤维化患者的临床特征及预后研究
- Author:
Zefeng XU
1
;
Tiejun QIN
;
Hongli ZHANG
;
Liwei FANG
;
Naibo HU
;
Lijuan PAN
;
Shiqiang QU
;
Bing LI
;
Xin YAN
;
Zhongxun SHI
;
Huijun HUANG
;
Dan LIU
;
Ya’nan CAI
;
Yudi ZHANG
;
Peihong ZHANG
;
Zhijian XIAO
Author Information
1. Institute of Hematology and Blood Diseases Hospital, CAMS & PUMC, The State Key Laboratory of Experimental Hematology, Tianjin 300020, China
- Publication Type:Journal Article
- Keywords:
Primary myelofibrosis;
Thrombocytopenia;
Clinical characteristic;
Prognosis
- From:
Chinese Journal of Hematology
2019;40(1):12-16
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To evaluate clinical characteristics and prognosis of primary myelofibrosis (PMF) patients with thrombocytopenia in varied degrees.
Methods:Clinical features and survival data of 1 305 Chinese patients with PMF were retrospectively analyzed. The prognostic value of thrombocytopenia in patients with PMF was evaluated.
Results:320 subjects (47%) presented severe thrombocytopenia (PLT<50×109/L), 198 ones (15.2%) mild thrombocytopenia [PLT (50-99)×109/L] and 787 ones (60.3%) without thrombocytopenia (PLT ≥ 100×109/L). The more severe the thrombocytopenia, the higher the proportions of HGB<100 g/L, WBC<4×109/L, circulating blasts ≥ 3%, abnormal karyotype and unfavourable cytogenetics (P<0.001, P<0.001, P=0.004, P<0.001 and P<0.001, respectively) were observed in this cohort of patients. The more severe the thrombocytopenia, the lower the proportion of JAK2V617F positive (P<0.001) was also noticed. Platelet count was positively correlated with splenomegaly, HGB and WBC (P<0.001, correlation coefficients were 0.131, 0.445 and 0.156, respectively). Platelet count was negative correlated with constitutional symptoms and circulating blasts (P=0.009, P=0.045, respectively; correlation coefficients were -0.096 and -0.056, respectively). The median survival of patients with severe thrombocytopenia, mild thrombocytopenia and without thrombocytopenia were 32, 67 and 89 months, respectively (P<0.001). Multivariate analysis identified thrombocytopenia in varied degrees (HR=1.693, 95%CI 1.320-2.173, P<0.001) and Dynamic Internation Prognostic Scoring System(DIPSS) prognostic model (HR=2.051, 95%CI 1.511-2.784, P<0.001) as independent risk factors for survival.
Conclusion:PMF patients with severe thrombocytopenia frequently displayed anemia, leucopenia, circulating blasts and short survival, so active treatment measures should be taken especially in these patients.
