185例真性红细胞增多症的临床分析

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。

Clinical analysis of 185 patients with polycythemia vera

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.