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原发性血小板增多症患者中根据JAK2 V617F突变的临床病理结果比较

Comparison of clinicopathologic findings according to JAK2 V617F mutation in patients with essential thrombocythemia.

作者信息

Cho Young-Uk, Chi Hyun-Sook, Lee Eun-Hye, Jang Seongsoo, Park Chan-Jeoung, Seo Eul-Ju

机构信息

Department of Laboratory Medicine, Eulji General Hospital, Eulji University School of Medicine, 280-1 Hagye-dong, Nowon-gu, Seoul, 139-872, South Korea.

Department of Laboratory Medicine, College of Medicine and Asan Medical Center, University of Ulsan, 388-1 Poongnap-dong, Songpa-gu, Seoul, 138-736, South Korea.

出版信息

Int J Hematol. 2009 Jan;89(1):39-44. doi: 10.1007/s12185-008-0222-0. Epub 2008 Dec 18.

Abstract

The JAK2 V617F mutation is present in most patients with polycythemia vera, but in fewer patients with essential thrombocythemia (ET). We have assessed the frequency of this mutation in ET patients using amplification refractory mutation system PCR and determined the relationship of the mutation with disease phenotypes. Clinical-laboratory findings and histomorphological features were compared according to mutational status in 108 ET patients. The mutation was detected in 61 patients (56.5%) including one homozygous patient. Those with the mutation had significantly higher leukocyte (P = 0.003) and neutrophil (P = 0.007) counts. However, the incidences of thrombotic events and progression to advanced stages did not differ significantly between patients with and without the mutation. Thrombotic events were significantly correlated with older age (P = 0.025). Morphological analysis revealed that erythroid hypoplasia was exclusively found in the mutation-negative patients (P = 0.027). We could confirm previous findings of higher leukocyte count in ET patients with JAK2 mutation, but could not find any correlation with thrombotic events. Therefore, the detection of the mutation could characterize a subset of ET patients with distinct phenotype, despite its clinical significance being still undetermined.

摘要

大多数真性红细胞增多症患者存在JAK2 V617F突变,但原发性血小板增多症(ET)患者中携带该突变的较少。我们使用扩增阻滞突变系统PCR评估了ET患者中该突变的频率,并确定了该突变与疾病表型的关系。根据108例ET患者的突变状态,比较了临床实验室检查结果和组织形态学特征。在61例患者(56.5%)中检测到该突变,其中包括1例纯合子患者。携带该突变的患者白细胞(P = 0.003)和中性粒细胞计数(P = 0.007)显著更高。然而,有突变和无突变患者的血栓形成事件发生率及进展至晚期的情况并无显著差异。血栓形成事件与年龄较大显著相关(P = 0.025)。形态学分析显示,红系造血减低仅见于突变阴性患者(P = 0.027)。我们能够证实既往关于JAK2突变的ET患者白细胞计数较高的发现,但未发现与血栓形成事件有任何相关性。因此,尽管该突变的临床意义仍未明确,但检测到该突变可对具有独特表型的一部分ET患者进行特征性描述。

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