Popescu Cyprian
Victor Pauchet Clinic, Amiens, France.
Case Rep Neurol. 2020 Jun 10;12(2):184-188. doi: 10.1159/000508356. eCollection 2020 May-Aug.
Herein, we report on a 44-year-old man who presented with cluster headache (CH)-like pain triggered by polycythemia vera (PV). He had severe unilateral head pain attacks lasting about 30 min not associated with cranial autonomic symptoms. After the exclusion of secondary etiologies, the patient was screened for a neoplastic process through biological markers, and the diagnosis of PV was established. The results of the initial laboratory examination showed hemoglobin at 18.1 g/L and Hct at 54%. JAK2 mutation analysis was positive at 54%, and marrow biopsy confirmed the hematopoietic clonal expansion, without myelofibrosis. He was treated with aspirin and ruxolitinib due to intolerance to interferon and the ineffectiveness of hydroxyurea. The treatment by venesection improved substantially the headaches. Oxygen inhalation was very effective in treating the CH attacks. In contrast, sumatriptan was inefficient at the very beginning of the disease. Among the pathophysiological mechanisms that we can propose to explain these cluster-like headaches are the prolonged hypoxia involving nitric oxide and calcitonin gene-related peptide release.
在此，我们报告一名44岁男性，其出现由真性红细胞增多症（PV）引发的丛集性头痛（CH）样疼痛。他有严重的单侧头痛发作，持续约30分钟，与颅神经自主症状无关。排除继发性病因后，通过生物标志物对患者进行肿瘤性疾病筛查，确诊为PV。初始实验室检查结果显示血红蛋白为18.1 g/L，血细胞比容为54%。JAK2突变分析阳性率为54%，骨髓活检证实造血克隆性增殖，无骨髓纤维化。由于对干扰素不耐受且羟基脲无效，他接受了阿司匹林和芦可替尼治疗。放血治疗使头痛症状大幅改善。吸氧对治疗CH发作非常有效。相比之下，舒马曲坦在疾病初期效果不佳。在我们可以提出的解释这些丛集样头痛的病理生理机制中，包括涉及一氧化氮和降钙素基因相关肽释放的长期缺氧。
