原发性血小板增多症40例临床分析

Clinical Analysis on 40 Cases of Essential Thrombocythemia

目的探讨原发性血小板增多症(ET)的临床表现、治疗及预后。方法对本院1994年5月至2007年12月间诊治的40例患者进行回顾性分析。结果40例患者中,男性28例,女性12例,中位年龄57岁(27~92岁)。临床表现血栓者8例(20.0%),脾大者6例(15.0%),出血者3例(7.50%),因其他原因化验血常规确诊本病者16例(40.0%),仅有头昏、乏力等非特异性症状者7例(17.5%)。就诊时中位血小板计数985×109/L(699×109/L^1200×109/L);40例均进行了骨髓活检,其中3例局部有网状纤维增生,Ph染色体、bcr/abl融合基因均阴性,4例检测JAK2/V617F阳性。随访28例,1例转变为骨髓纤维化,未发现转变为急性白血病和骨髓增生异常综合征的病例。23例羟基脲(Hu)和17例羟基脲+干扰素(IFN)治疗有效率分别为87.0%和94.1%。结论原发性血小板增多症患者临床表现以血栓、脾大和出血多见,以羟基脲或羟基脲+干扰素治疗可以获得较好疗效,预后较好。

Objective To explore the clinical manifestation, treatment and prognosis of essential thromboeytemia （ET）. Methods A retrospective analysis was conducted on 40 ET cases treated in this hospital during May,1994 to Dec,2007. Results 40 cases （28 males and 12 females with a median age of 57 years） were diagnosed, thrombosis occurred in 8 cases（20.0%） , hemorrhage occurred in 3 cases（7.50% ） , and 6 cases （ 15.0% ） seeking medical advice due to splenomegaly, 16 cases（ 40.0% ） confirmed by routine blood examination due to other causes, 7 cases（ 17.5% ） exhibiting only such nonspecific symptoms as dizziness or fatigue. The median thrombocyte count at diagnosis was 985 × 10^9/L （range from 699× 10^9/L to 1200 × 10^9/L ）. Bone marrow biopsy was performed in all cases and local fibrosis was revealed in 3 cases; Ph chromosome and BCR/ABL were negative in all cases, 4 cases were positive for JAK2/V617F. Among 28 followed - up cases, one patient evolved into marrow fibrosis, and other patients are stable. The effective rates of 23 patients treated with hydmxyurea （Hu） and 17 patients treated with Hu combined with interferon （ IFN ） were 88% and 95% respectively. Conclusions The main clinical manifestations of essential thrombocythemia were thrombosis and hemorrhage. A good therapeutic effect can be achieved by treatment of hydroxyurea or hydroxyurea combined with interferon with favorable prognosis.