Predictive values of X-chromosome inactivation patterns and clinicohematologic parameters for vascular complications in female patients with essential thrombocythemia.
Predictive values of X-chromosome inactivation patterns and clinicohematologic parameters for vascular complications in female patients with essential thrombocythemia.
复制标题
X染色体失活模式和临床血液学参数对原发性血小板增多症女性患者血管并发症的预测价值。
作者：
L. Shih;Tung‐Liang Lin;C. Lai;P. Dunn;Jin‐Hou Wu;Po‐Nan Wang;M. Kuo;L. Lee
原发性血小板增多症（ET）是一种异质性疾病，其中造血的克隆性不同。ET克隆状态的临床意义仍有待确定。我们采用人雄激素受体基因(HUMARA)-聚合酶链反应法对89例ET女性患者的x染色体失活模式（XCIP）及其对血管并发症的预测价值进行了研究。54例（68.4%）患者为XCIP克隆型，15例（19.0%）患者为XCIP多克隆型。其余20例患者有不明确或纯合的XCIP模式，因此被排除在进一步的分析之外。克隆XCIPs患者比多克隆XCIPs患者年龄更大（P = 0.029），血栓形成风险更高（P = 0.007）。我们没有发现出血的发生与XCIP之间的相关性（P =.492）。高龄可预测血栓和出血。血小板计数不影响血管并发症的发生。高血压与血栓形成事件显著相关（P = 0.002），而糖尿病和高胆固醇血症没有预测价值。在多变量分析中，年龄是血栓形成的显著预测因子（P = 0.030）；而XCIPs （P = 0.083）和高血压（P = 0.073）倾向于预测血栓形成。我们的研究结果表明，患有克隆性xcip或高血压的老年患者血栓形成的风险增加，应密切监测这种并发症。
Essential thrombocythemia (ET) is a heterogeneous disorder in which the clonality of hematopoiesis varies. The clinical significance of clonality status in ET remains to be determined. We used the human androgen receptor gene (HUMARA)-polymerase chain reaction assay to investigate X-chromosome inactivation patterns (XCIPs) and their value in predicting vascular complications in 89 female patients with ET. Fifty-four (68.4%) patients had a clonal pattern of XCIP, and 15 (19.0%) had a polyclonal pattern. The remaining 20 patients had either an ambiguous or a homozygous pattern of XCIP and were therefore excluded from further analysis. Patients with clonal XCIPs were older (P =.029) and were at greater risk for thrombosis (P =.007) than were those with polyclonal XCIPs. We did not find a correlation between the occurrence of hemorrhage and XCIP (P =.492). Advanced age was predictive of thrombosis and hemorrhage. Platelet count did not influence the risk for vascular complications. Hypertension was significantly correlated with thrombotic events (P =.002), whereas diabetes mellitus and hypercholesterolemia were of no predictive value. In a multivariate analysis, age was the significant predictor of thrombosis (P =.030); however, XCIPs (P =.083) and hypertension (P =.073) tended to predict thrombosis. Our results suggest that older patients who have clonal XCIPs or hypertension are at increased risk for thrombosis and should be monitored closely for this complication.