JAK2 mutation?positive polycythaemia vera associated with IgA vasculitis and nephrotic syndrome: a case report
JAK2 mutation?positive polycythaemia vera associated with IgA vasculitis and nephrotic syndrome: a case report
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JAK2突变阳性真性红细胞增多症与IgA血管炎和肾病综合征相关：一例报告
DOI：
10.1080/24725625.2020.1728061
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发表时间：
2020
影响因子：
0.8
通讯作者：
Fujii Hiroshi
中科院分区：
文献类型：
--
作者：
Kondo Hinako;Watanabe Ryu;Okazaki Soshi;Kuriyama Kaori;Ochi Tetsuro;Yamada Gen;Sugiura Akira;Chiba Hiromu;Tsukada Akira;Taniuchi Shinji;Igarashi Takehiko;Kudo Masataka;Harigae Hideo;Fujii Hiroshi
我们报告一例真性红细胞增多症（PV）与伊加血管炎。一位45岁男性因腹痛和可触及的紫癜而入院。诊断为伊加血管炎，开始口服泼尼松龙治疗（30 mg/天）。第6天，患者出现左侧轻偏瘫，磁共振成像显示急性脑梗死。骨髓活检结果和Janus激酶2（JAK 2）突变的鉴定导致PV的诊断。尽管类固醇治疗，尿蛋白水平增加到15 g/g·Cre。肾活检显示轻度系膜增生伴伊加沉积，但免疫抑制治疗部分有效。该病例表明PV可能是伊加血管炎的并发症，在此类病例中应采取血栓形成的预防措施。
We report a case of polycythaemia vera (PV) associated with IgA vasculitis. A 45-year-old man was admitted for evaluation of abdominal pain and palpable purpura. IgA vasculitis was diagnosed, and oral prednisolone therapy (30 mg/day) was initiated. On day 6, the patient developed left hemiparesis, and magnetic resonance imaging revealed acute cerebral infarction. Bone marrow biopsy results and the identification of a Janus kinase 2 (JAK2) mutation led to the diagnosis of PV. Despite steroid therapy, urine protein levels increased to 15 g/g・Cre. Renal biopsy demonstrated mild mesangial proliferation with IgA deposits, but immunosuppressive therapy was partially effective. This case suggests that PV can be a complication of IgA vasculitis and that preventive measures for thrombosis should be taken in such cases.