原发性血小板增多症的临床特点(附60例分析)  

Clinical characteristics of with essential thrombocythemia:a case study of 60 patients

作　　者：许蕾[1] 庞丽萍[1] 温娟娟[1] 徐海婵[1] 孙丽华[1] 覃宝珍[1] 

机构地区：[1]北京大学深圳医院血液内科,深圳518036

出　　处：《右江医学》2014年第3期288-291,共4页Chinese Youjiang Medical Journal

摘　　要：目的探讨原发性血小板增多症（essential thrombocythemia,ET）的临床特点及病程转归。方法回顾分析2003年7月至2013年3月间诊治的60例ET患者的临床资料,并总结疾病的自然病程。结果 60例患者中,男性38例,女性22例,发病年龄21～90岁,中位发病年龄45.5岁,有出血症状者12例（20.0%）,血栓症状者15例（25.0%）,同时有出血及血栓者3例（5.0%）,无症状者30例（50.0%）。脾肿大35例（58.3%）,肝大4例（6.7%）。初诊时血小板计数（522～2384）×109/L,中位血小板计数1015×109/L,同时有白细胞升高者50例（83.3%）,血红蛋白升高者20例（33.3%）。51例患者做了骨髓活检,以大而多分叶的成熟巨核细胞增生为主,其中8例（15.7%）伴有网状纤维增生。48例行染色体核型检查,3例（6.3%）有异常。38例行JAK2V617F突变检测,30例（78.9%）阳性,8例（21.1%）阴性。随访时间12～48个月,中位随访时间28个月,3例（6.7%）发展为骨髓纤维化（myelofibrosis,MF）,1例转化为慢性中性粒细胞白血病,其余病情稳定。结论 ET是一种慢性骨髓增殖性疾病,病程发展缓慢,临床症状以血栓和出血为主,无症状者比例较高,大多预后较好,主要进展为骨髓纤维化。Objective To explore the clinical characteristics and prognosis of essential thrombocythemia（ET）.Methods A retrospective analysis was conducted to the clinical data of 60 ET patients diagnosed and treated in our hospital during July,2003 and March,2013.The natural course of the disease was concluded.Results Among the sixty diagnosed patients,there were 38 males and 22 females with a median age of 45.5 and ages ranged from 21-90 were diagnosed. Hemorrhage occurred in 12 cases （20 % ） ; thrombosis in 15 cases （ 25 % ） ; both hemorrhage and thrombosis in 3 cases（ 5 % ）; no symptoms in 30 cases. Splenomegaly occurred in 35 eases（58.3 % ） and hepatomegaly occurred in 4 cases （6.7 % ）.At diagnosis, the platelet count was （522-2384）×109/L and the median platelet count was 1015Х 109/L.Fif- ty cases（83.3 %）had white blood cell increased and twenty cases（33.3 %）had hemoglobin higher. 52 cases underwent bone marrow biopsy which showed mainly the hyperplasia of mature enlarged megakaryocytes and 8（15.7% ） of them were accompanied with proliferation of reticular fiber.Karyotype analysis was conducted in forty eight cases and three cases（6.3%）had elonal chromosomal aberrations. JAK2V617F mutation was detected in 30 （78.9%） of 38 cases.Sixty patients were followed up for over twelve months with a median follow-up of twenty eight months （range twelve to forty-eight months）.Three cases（6.7 %） evolved into marrow fibrosis （MF） and one case into chronic neutrophils leu- kemia.The others had stable course.Conclusion ET is a chronic myeloproliferative disease characterized predominate- ly by thrombosis and hemorrhage.The percentage of asymptomatic cases is high.The prognosis of most cases are bet- ter,but a few cases may evolve into MF.

关 键 词：血小板增多症 原发性 临床特点 病程转归 

分 类 号：R558.3[医药卫生—血液循环系统疾病]