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伴有Jak 2 V671F阳性原发性血小板增多症的Zap-70阳性慢性淋巴细胞白血病:一种常见的缺陷干细胞?

Zap-70 positive chronic lymphocytic leukemia co-existing with Jak 2 V671F positive essential thrombocythemia: a common defective stem cell?

作者信息

Tabaczewski Piotr, Nadesan Sushani, Lim Seah H

机构信息

Department of Internal Medicine, Texas Tech University Health Sciences Center, Amarillo, United States.

出版信息

Leuk Res. 2009 Jun;33(6):854-5. doi: 10.1016/j.leukres.2008.09.002. Epub 2008 Oct 11.

Abstract

Essential thrombocythemia (ET) co-existing with chronic lymphocytic leukemia (CLL) is extremely rare. We report two cases of ET with Jak 2 V617F in Zap-70+ CLL. ET is a myeloproliferative stem cell disease. Zap-70 expression in CLL correlates with non-mutated immunoglobulin genes. The occurrence of a less mature CLL in patients with a pluripotential stem cell disease raises the possibility that an initial "trigger hit" occurred in a pre-Jak 2 common early progenitor in these patients. Subsequent additional molecular events accumulated independently following myeloid and lymphoid differentiation, leading to the development of two diseases of likely identical origin but different lineages.

摘要

原发性血小板增多症(ET)与慢性淋巴细胞白血病(CLL)共存极为罕见。我们报告了两例Zap-70阳性的CLL合并Jak 2 V617F突变的ET病例。ET是一种骨髓增殖性干细胞疾病。CLL中Zap-70的表达与未突变的免疫球蛋白基因相关。在多能干细胞疾病患者中出现较不成熟的CLL,提示这些患者可能在Jak 2之前的共同早期祖细胞中发生了初始的“触发打击”。随后,在髓系和淋巴系分化后,额外的分子事件独立积累,导致了两种可能起源相同但谱系不同的疾病的发生。

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