医学
真性红细胞增多症
鲁索利替尼
血液学
骨髓增生性肿瘤
内科学
红细胞压积
骨髓纤维化
Janus激酶2
羟基脲
一线治疗
静脉切开术
肿瘤科
骨髓
重症监护医学
疾病
化疗
受体
出处
期刊:Annals of Hematology
[Springer Science+Business Media]
日期:2023-03-21
卷期号:102 (5): 985-993
被引量:3
标识
DOI:10.1007/s00277-023-05172-y
摘要
Abstract Polycythemia vera (PV) is a burdensome, chronic myeloproliferative neoplasm characterized by activating mutations in Janus kinase 2, erythrocytosis, and bone marrow hypercellularity. The goals of treatment are to achieve hematocrit and blood count control to ultimately reduce the risk of thrombohemorrhagic events and improve PV-related symptoms. Treatment is risk-stratified and typically includes cytoreduction with hydroxyurea or interferon formulations in first line for high-risk disease. However, inadequate response, resistance, or intolerance to first-line cytoreductive therapies may warrant introduction of second-line treatments, such as ruxolitinib. In this review, I detail preferred treatment and patient management approaches following inadequate response to or intolerance of first-line treatment for PV.
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