原发性血小板增多症98例临床特点研究

Study on clinical characteristics of essential thrombocytopenia in 98 cases

目的分析原发性血小板增多症(ET)患者的临床特征和预后。方法收集2013年4月至2017年4月该院血液内科收治的114例ET患者的临床资料,纳入病历资料完整的98例,对不同基因突变患者的发病年龄、临床表现、血液学特征进行回顾性分析。结果与JAK2V617F突变阴性患者比较,JAK2V617F突变阳性患者具有诊断时年龄更大、血红蛋白更高、白细胞计数更高、血小板计数更低等特点,但在随访过程中二者心血管并发症、血栓形成、出血等并发症发生率比较,差异均无统计学意义(P>0.05)。与钙网蛋白(CALR)阴性组比较,CALR阳性组患者血小板计数更高、白细胞数更低,差异均有统计学意义(P<0.05)。结论基因突变检测对ET的诊断具有重要意义,不同基因突变ET患者临床和血液学特征不同,但对预后的影响尚需进一步研究证实。

Objective To analyze the clinical features and prognosis of the patients with essential thrombocytopenia（ET）. Methods The clinical data in 114 cases of ET in the department of hematology of this hospital from April 2013 to April2017 were collected,in which 98 cases of intact medical record data were included. The onset age,clinical manifestations and hematological features in the patients with different gene mutations were retrospectively analyzed. Results Compared with the cases of JAK2 V617 F mutation negative,the cases of JAK2 V617 F mutation positive had the characteristics of older age,higherhemoglobin,higher WBC count and lower platelet count,but two groups′ the occurrence rates of complications such as cardiovascular complications,thrombosis and hemorrhage during follow-up period had no statistical difference（P〉0.05）. Comparedwith the CALR negative group,the CALR positive group had higher platelet count and lower WBC count,the difference was statistically significant（P〈0.05）. Conclusion The genetic mutation detection is of great significance for the diagnosis of ET. Clinical and hematological features are different among ET patients with different gene mutations,and its effects on prognosis need to be confirmed further.