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JAK2 突变阳性的真性红细胞增多症伴 IgA 血管炎和肾病综合征:一例报告。

JAK2 mutation-positive polycythaemia vera associated with IgA vasculitis and nephrotic syndrome: a case report.

机构信息

Department of Rheumatology, Osaki Citizen Hospital, Osaki, Japan.

Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Sendai, Japan.

出版信息

Mod Rheumatol Case Rep. 2020 Jul;4(2):289-295. doi: 10.1080/24725625.2020.1728061. Epub 2020 Feb 13.

Abstract

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 () 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.

摘要

我们报告一例与 IgA 血管炎相关的真性红细胞增多症 (PV)。一名 45 岁男性因腹痛和可触及性紫癜入院。诊断为 IgA 血管炎,并开始口服泼尼松龙治疗(30mg/天)。第 6 天,患者出现左侧偏瘫,磁共振成像显示急性脑梗死。骨髓活检结果和 Janus 激酶 2 () 突变的鉴定导致 PV 的诊断。尽管进行了类固醇治疗,尿蛋白水平仍增加至 15g/g・Cre。肾活检显示轻度系膜增殖伴 IgA 沉积,但免疫抑制治疗部分有效。本病例提示 PV 可作为 IgA 血管炎的并发症,此类情况下应采取预防血栓形成的措施。

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