185例真性红细胞增多症的临床分析

Clinical analysis of 185 patients with polycythemia vera

目的 了解真性红细胞增多症 (PV)患者临床特点及疾病自然过程。方法 分析 185例PV患者临床症状、体征、实验室检查及转归。结果 185例患者中 ,男 12 2例 ,女 6 3例。发病年龄 (5 2 .7± 14 .1)岁。初诊时血红蛋白为 (2 0 8.3± 2 1.2 )g/L。血常规三系增高 74例 (40 .0 % ) ,单纯血红蛋白增高 33例 (17.8% ) ,血红蛋白增高伴有白细胞增高 6 7例 (36 .2 % ) ,血红蛋白增高伴有血小板增高 11例(5 9% )。脾大 12 3例 (6 6 .5 % ) ,肝大 3例 (16 .2 % )。 2 5例患者检测Epo水平 ,9例 (36 .0 % )正常 ,16例 (6 4 .0 % )减低。 30例患者进行干 /祖细胞培养 ,5 7%CFU E增高 ,5 9%BFU E增高 ,其中 11例检测内源性红系集落 (EEC) ,10例 (90 .9% )出现EEC。 82例 (44 .3% )发生血栓栓塞 10 3次 ,5例进展为骨髓纤维化 (MF) ,1例发生继发肿瘤 ,2例死于栓塞。结论 PV是一种成年型骨髓增殖性疾病 ,极易并发栓塞 ,易检出EEC ,且血清Epo水平多减低 ,主要进展为MF。

Objective To understand the clinical feature and natural course of polycythemia vera(PV). Methods The clinical symptoms, signs, laboratory examination and prognosis of 185 patients with PV were analysed. Results There are 122 males and 63 females. The mean age was (52.7±14.1) years. The mean hemoglobin level was (208.3±21.2)g/L. Pancytosis was displayed in 74 (40%) cases, excess of red blood cells in 33(17 8%), excess of red blood cells and granulocytes in 67(36.2%) and excess of red blood cell and platelets in 11(5 9%). Splenomegaly was found in 123 (66.5%) patients and hepatomegaly in 30(16.2%). Quantitative assess of serum Epo was done in 25 patients. The level was low in 16(64.2%) and normal in 9(36.0%). Hematopoietic progenitor culture yields was elevated in 11 patients, endogenous erythroid colonies (EEC) formation was found in 10 cases (90.9%). Eighty two patients (44.3%) had 101 attacks of vascular thrombotic incidents, 7 patients developed myelofibrosis(MF). Secondary cancer occurred in 1 patient. Two patients died of thrombosis. Conclusion PV is an elderly adult myeloproliferative disease with a high frequency of thrombosis. EEC can be found out in PV patients. The serum Epo level is not increased in PV patients. The main sequelae of PV is MF.