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伴嗜酸粒细胞增多的骨髓增生性肿瘤伴 t(8;9)(p21;p24)易位患者对芦可替尼的持续缓解。

Sustained Response to Ruxolitinib of Eosinophilia-Associated Myeloproliferative Neoplasm with Translocation t(8;9)(p21;p24).

机构信息

National Clinical Research Center for Hematologic Diseases, Jiangsu Institute of Hematology, NHC Key Laboratory of Thrombosis and Hemostasis, The First Affiliated Hospital of Soochow University, Suzhou, China.

Department of Hematology, NanYang Central Hospital, Henan, China.

出版信息

Acta Haematol. 2023;146(5):397-400. doi: 10.1159/000510281. Epub 2023 Aug 10.

Abstract

The translocation t(8;9) produces the fusion gene PCM1-JAK2, resulting in the continuous activation of the JAK2 tyrosine kinase. Myelodysplastic/myeloproliferative neoplasms are the most common disease with t(8;9)/PCM1-JAK2. Individuals with this abnormality have similar features, and JAK2 kinase inhibitor (ruxolitinib) is an effective treatment of the condition. The long-term remission results of ruxolitinib are varied. It is important to determine the response to ruxolitinib. Here, we describe a patient who has been diagnosed with eosinophilia-associated myeloproliferative neoplasm with t(8;9)(p21;p24). This patient has achieved sustained response for >1 year since the administration of ruxolitinib.

摘要

t(8;9)易位导致 PCM1-JAK2 融合基因的产生,从而导致 JAK2 酪氨酸激酶的持续激活。骨髓增生异常/骨髓增殖性肿瘤是最常见的疾病,伴有 t(8;9)/PCM1-JAK2。具有这种异常的个体具有相似的特征,JAK2 激酶抑制剂(鲁索利替尼)是该疾病的有效治疗方法。鲁索利替尼的长期缓解结果各不相同。确定对鲁索利替尼的反应非常重要。在这里,我们描述了一名被诊断为伴有 t(8;9)(p21;p24)的嗜酸粒细胞相关骨髓增殖性肿瘤的患者。该患者自服用鲁索利替尼以来,已持续缓解>1 年。

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