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鲁索替尼治疗真性红细胞增多症患者时肢端发绀的可逆性及毛细血管镜检查结果的改善:一例报告

Reversibility of acrocyanosis and improvement of capillaroscopic pattern in a patient with polycythemia vera treated with ruxolitinib: a case report.

作者信息

Nigro Angelo

机构信息

Department of Rheumatology of Lucania - UOSD of Rheumatology, "Madonna Delle Grazie" Hospital, Matera, Italy.

出版信息

Blood Res. 2025 Jan 30;60(1):8. doi: 10.1007/s44313-024-00053-3.

Abstract

This case report describes the clinical course of a 78-year-old patient diagnosed with polycythemia vera (PV), who presented with pronounced acrocyanosis of the hands in 2021. The patient was treated with hydroxyurea (oncocarbide), and nailfold capillaroscopy revealed an "abnormal pattern" characterized by pronounced architectural disarray and capillary tortuosity, which is uncommon in patients with myeloproliferative neoplasms (MPNs). In 2023, owing to suboptimal symptom management and hematological side effects, the treatment was switched to ruxolitinib, which led to significant clinical improvements by 2024, including near-complete resolution of acrocyanosis and substantial improvement in capillaroscopic abnormalities, with only residual capillary tortuosity noted. This case emphasizes the need for individualized therapeutic interventions for PV, and underscores the potential role of ruxolitinib in ameliorating microvascular dysfunction.

摘要

本病例报告描述了一名78岁诊断为真性红细胞增多症(PV)患者的临床病程,该患者在2021年出现明显的手部肢端发绀。患者接受了羟基脲(奥可巴胺)治疗,甲襞毛细血管镜检查显示出一种“异常模式”,其特征为明显的结构紊乱和毛细血管迂曲,这在骨髓增殖性肿瘤(MPN)患者中并不常见。2023年,由于症状管理欠佳和血液学副作用,治疗改为鲁索替尼治疗,并在2024年取得了显著的临床改善,包括肢端发绀几乎完全消退以及毛细血管镜异常显著改善,仅观察到残留的毛细血管迂曲。本病例强调了PV个体化治疗干预的必要性,并突出了鲁索替尼在改善微血管功能障碍方面的潜在作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c3d1/11782742/40bc93a8e5d4/44313_2024_53_Fig1_HTML.jpg

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