Bai Jie, Shao Zonghong, Jing Liping, Liu Hong, Shi Jun, Zhao Mingfeng, Fu Rong, He Guangsheng, Sun Juan, Jia Hairong, Qian Linsheng, Yang Tianying, Yang Chongli
Institute of Hematology and Blood Diseases Hospital, CAMS and PUMC, Tianjin 300020, China.
Zhonghua Xue Ye Xue Za Zhi. 2002 Nov;23(11):578-80.
To understand the clinical feature and natural course of polycythemia vera (PV).
The clinical symptoms, signs, laboratory examination and prognosis of 185 patients with PV were analysed.
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.
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.
了解真性红细胞增多症(PV)的临床特征及自然病程。
分析185例PV患者的临床症状、体征、实验室检查及预后情况。
男性122例,女性63例。平均年龄为(52.7±14.1)岁。平均血红蛋白水平为(208.3±21.2)g/L。74例(40%)表现为全血细胞增多,33例(17.8%)为红细胞增多,67例(36.2%)为红细胞和粒细胞增多,11例(5.9%)为红细胞和血小板增多。123例(66.5%)患者有脾肿大,30例(16.2%)有肝肿大。对25例患者进行了血清促红细胞生成素(Epo)定量检测。16例(64.2%)水平较低,9例(36.0%)正常。11例患者造血祖细胞培养产率升高,10例(90.9%)发现内源性红系集落(EEC)形成。82例(44.3%)患者发生101次血管血栓事件,7例发展为骨髓纤维化(MF)。1例发生继发性癌症。2例死于血栓形成。
PV是一种老年成人骨髓增殖性疾病,血栓形成频率高。PV患者可检测到EEC。PV患者血清Epo水平不升高。PV的主要后遗症是MF。