原发性血小板增多症-75例临床分析

Essential Thrombocytemia: Clinical Analysis of 75 Cases

目的探讨原发性血小板增多症(ET)的临床表现、诊断、治疗及预后。方法对本院1982年5月至2004年4月间诊治的75例ET患者进行回顾性分析。结果男:女=2.6∶1。就诊时中位年龄53岁(17-86岁),有出血者28例(37.3%),有栓塞者21例(28%);因脾大就诊者20例(26.6%);有肝大者13例(13.3%);因其它原因做血常规确诊本病者10例(13.3%);仅有头昏、乏力等非特异性症状者8例(10.6%)。就诊时中位血小板数1 205×109/L (800-2 898×109/L)。骨髓象见巨核细胞明显增生,以产板型与颗粒型为主。45例做骨髓活检,以巨核细胞增生为主,呈异位集簇现象,3例显示网状纤维增生。羟基脲(Hu)治疗有效率(CR+PR)达88%,Hu+干扰素(IFN)治疗有效率达94%。随访18例(1-10年),有5例就诊年龄<40岁者,经Hu治疗半年后不需继续治疗,13例仍在间断治疗中, 病情稳定,2例转为骨髓纤维化,无1例转为AML或MDS者。结论 ET出血与栓塞的发生率相仿。以Hu或Hu+IFN 的治疗可获较好疗效。预后良好。

Objective To investigate the clinical manifestation,diagnosis,treatment and prognosis of essential thrombocytemia( ET). Methods A retorspective analysis was conducted of 75 ET cases treated from May, 1982 to April,2004 in the hospital. Results The median age of the 75 cases (male/female ratio 2. 6: 1 ) on being diagnosed was 53 years (17 -86 years) ,of whom 28 cases (37. 3% ) with hemorrhage,21 cases (28% ) with embolism,20 cases (26. 6% ) seeking medical advice due to splenomegaly ,13 cases (17. 3% ) due to hepatomegaly, and 10 cases ( 13. 3% ) confirmed by routine blood teses due to-other reasons, 8 cases (10. 6% ) exhibiting only such nonspecific symptoms as dizziness or asthenia. The median thrombocyte count when the cases were being diagnosed was 1 205× 109 /L(800 -2 898× 10 /L). Myelogram showed remarkable megakaryocyte proliferation mainly of platelet-forming type and granular type. Bone marrow biopsy was performed in 45 cases showing mainly megakaryocyte proliferation with ectopic aggregation, and 3 cases showing proliferation of reticular fibers. Effective rate( CR + PR) of those treated with hydroxyurea( Hu) was 88% ,and that with Hu + IFN( interferon) was 94% . Of the 18 follow-up cases (1-10 years) ,5 ( less 40 years old when being treated) were free from treatment after halt a year of Hu therapy, 13 were still under discontinuous treatment with stable condition ;2 cases were converted into myelofibrosis but none into AML or MDS. Conclusion The data suggest that the incidence of hemorrhage and that of embolism in ET is similar, and a good therapeutic effect can be achieved with treatment of Hu of Hu + IFN with favorable prognosis.