Department of Rheumatology, Osaki Citizen Hospital, Osaki, Japan.
Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Sendai, Japan.
Mod Rheumatol Case Rep. 2020 Jul;4(2):289-295. doi: 10.1080/24725625.2020.1728061. Epub 2020 Feb 13.
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.
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。
