JAK2 mutation–positive polycythaemia vera associated with IgA vasculitis and nephrotic syndrome: a case report. (2nd July 2020)

• Record Type: Journal Article
• Authors: 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
• Is Part Of: Modern rheumatology case reports. Volume 4:Number 2(2020)
• Page Start: 289
• Page End: 295
• Publication Date: 2020-07-02
• Subjects: IgA vasculitis -- Janus kinase 2 (JAK2) -- myeloproliferative neoplasms -- nephrotic syndrome -- polycythaemia vera; Rheumatology -- Periodicals; 616.723005
• DOI: 10.1080/24725625.2020.1728061
• Languages: English
• ISSNs: 2472-5625

Abstract

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 ( 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.