Li Li, Zhou Min, Wu Yun-Qin, Fan Wei-Nv, Li Da
Department of Neurology, Ningbo No 2 Hospital, Ningbo, Zhejiang, China.
Front Neurol. 2023 Sep 22;14:1253468. doi: 10.3389/fneur.2023.1253468. eCollection 2023.
Neuropsychiatric disturbances and chorea are less recognized consequences of polycythemia vera (PV), and their role in post-PV myelofibrosis (MF) has not been reported. Clinical features that predict post-PV MF lack specificity.
We describe an elderly patient with PV who developed acute-onset reversible neuropsychiatric disturbances accompanied by generalized chorea and was finally diagnosed with post-PV MF after a bone marrow examination. We also reviewed four cases of late PV associated with neuropsychiatric symptoms since 1966 and analyzed their clinical characteristics and therapeutic effects.
Our case indicates that Janus kinase 2 (JAK2)-related PV is a treatable cause of late-onset chorea and that chorea may herald the deterioration of hematological parameters. Our case provides a clinically specific representation of post-PV MF. Patients with a long course of PV are recommended to undergo bone marrow re-examinations when they present with neuropsychiatric symptoms to achieve an early diagnosis of post-PV MF.
神经精神障碍和舞蹈症是真性红细胞增多症(PV)较少被认识到的后果,其在PV后骨髓纤维化(MF)中的作用尚未见报道。预测PV后MF的临床特征缺乏特异性。
我们描述了一名患有PV的老年患者,该患者出现急性起病的可逆性神经精神障碍并伴有全身性舞蹈症,最终经骨髓检查确诊为PV后MF。我们还回顾了自1966年以来4例与神经精神症状相关的晚期PV病例,并分析了其临床特征和治疗效果。
我们的病例表明,Janus激酶2(JAK2)相关的PV是迟发性舞蹈症的可治疗病因,且舞蹈症可能预示血液学参数的恶化。我们的病例提供了PV后MF的临床特异性表现。建议病程较长的PV患者出现神经精神症状时进行骨髓复查,以早期诊断PV后MF。