原发性血小板增多症42例临床分析

Clinical Analysis of 42 cases of Essential Thrombocythemia

目的 :探讨原发性血小板增多症 (ET)的临床表现、诊断及预后。方法 :对 42例ET患者进行回顾性分析。结果 :因其他原因作血液检查被诊断 12例 (2 8.6% ) ,有出血症状 18例 (4 2 .9% ) ,有明确血栓症状 7例 (16.7% )。脾肿大 2 9例 (69.1% ) ,肝肿大 15例 (3 5 .7% )。BPC平均 12 0 0× 10 9/L。骨髓象 :巨核细胞明显增多 ,分类以产板型及裸核型为主 ,2 8例作骨髓活检 ,以巨核细胞增生为主 ,并呈异位集簇现象 ,18例显示网状纤维增生。 2 9例血小板聚集试验 ,2 5例正常 ,4例降低。随访 2 0例 ,中位随访时间 8年 (1～ 2 2年 ) ,4例转为骨髓纤维化 ,3例并发骨髓增生异常综合征 ,未发现 1例转化为白血病 ,与本病相关死亡 8例。结论 :马利兰、羟基脲均有良好疗效 。

Objective:To observe the clinical findings and the prognosis of patients with Essential Thrombocythemia(ET).Methods:ETpatients diagnosis in our hospital during Jaunary 1978 and Jaunary 2001 were analyzed.Results:42 patients were diagnosed in this period. Patients without clinical symptom occurred in 28.6%,hemorrhage , in 42.9%,thrombosis in 16.7%.splenomegaly,in 69.1%,hepatomegaly,in 35.7%. Onset of illness,the median platelet count at diagnosis was 1200×10 9/L. Bone marrow smear:megakaryocytes was significantly increased.Bone marrow biopsy was performed in 28 cases , increased megakaryocytes with ectopic concentration in all cases and local reticular fibrosis was revealed in 18 cases. .Platelet aggregation was performed in 29 cases,25 cases were normal,4 cases were lower.20 patients were follow-up with a median follow-up period of 8 years(range from 1 to 22 years),4 cases evolved into marrow fibrosis,3 cases into MDS,no cases into leukemia.Death associated ET in 8 cases.Both of busulphan or hydroxyure had a siginificantly effective. Conclusions:Busulphan or hydroxyure have an obvious effect on ET.The prognosis is relatively good.