原发性血小板增多症34例临床分析  

Clinical Analysis of 34 Cases With Essential Thrombocythemia

作　　者：王芳[1] 罗莉[1] 黄丽芳[1] 吴学琼[1] 邓金牛[1] 黄梅[1] 孙汉英[1] 

机构地区：[1]华中科技大学同济医学院附属同济医院,武汉430030

出　　处：《内科急危重症杂志》2009年第2期90-92,共3页Journal of Critical Care In Internal Medicine

摘　　要：目的:了解原发性血小板增多症(ET)患者临床特点,以减少误诊及并发症。方法:对34例ET患者的临床资料进行回顾性分析。结果:34例中,男13例,女21例,中位发病年龄51岁。多以头晕、头痛、肢体麻木为主诉;无特异性症状,体检发现PLT显著升高3例;因脑梗死、心肌梗死等住院发现PLT显著升高7例;因肠系膜上静脉、脾静脉、门静脉血栓形成表现腹胀、腹痛5例;因脾脏轻度肿大行切脾术3例。有出血症状者3例(8.8%),有血栓形成者11例(32.4%),同时有血栓形成和出血者2例(5.9%)。初诊时中位PLT为991×109/L。25例进行骨髓活检,骨髓增生活跃或明显活跃,以胞体大、核分叶多的巨核细胞增多为主。12例检测了JAK2V617F基因,阳性3例。经羟基脲(Hu)治疗有效率(缓解+进步)达85.7%,Hu+α-干扰素(IFN-α)治疗有效率达90%。结论:ET无特异临床表现,有脑梗死、心肌梗死、腹痛、脾脏增大、血栓形成表现者,查PLT升高达600×109/L时需警惕ET可能,尽早确诊,避免并发症。Objective： To understand the clinical features of essential thrombocythemia（ET）, in order to reduce misdiagnosis and avoid complications. Methods： Clinical data of 34 patients with ET were analyzed retrospectively. Results .. Among 34 patients, 13 were males and 21 were females with a median age of 51 years old. Most symptomat ic patients presented with dizziness, headache, limb paresthesias; 3 cases were asyrnptomatic, confirmed by routine blood test in health examination;7 cases were confirmed by blood routinein course of cerebral infarction or myocardial infarction; 5 cases presented abdominal distension or bellyache due to thrombosis of superior mesenterie vein, splenic vein or portal vein;3 cases with mild splenomegaly and underwent splenectomy. Hemorrhage occurred in 3 cases（8. 8%） ,thrombosis in 11 cases（32.4%）, and both hemorrhage and thrombosis in 2 cases （5.9%）. The median platelet count at initial diagnosis was 991 ×10^9/L. Bone marrow biopsy was performed in 25 cases which showed bone marrow proliferating actively or apparent active, mainly increase of enlarged megakaryocytes with hyperlobu lated nuclei. JAK2V617F mutation was detected in 3 of 12 patients. 85.7% of patients responsed to hydroxyurea, 90% of patients were responsive to hydroxyurea and interferon -α. Conclusions： For most symptomatic patients presenting with cerebral infarction, myocardial infarction, bellyache, splenomegaly or thrombosis, vigilance should be alert for the possibility of ET when blood routine showed PLT higher than 600 ×10^9/L. Early diagnosis may prevent complications.

关 键 词：血小板增多症 原发性 临床分析 

分 类 号：R558.3[医药卫生—血液循环系统疾病] R657.3[医药卫生—内科学]