JAK2 mutation-positive polycythaemia vera associated with IgA vasculitis and nephrotic syndrome: a case report.
Hinako KondoRyu WatanabeSoshi OkazakiKaori KuriyamaTetsuro OchiGen YamadaAkira SugiuraHiromu ChibaAkira TsukadaShinji TaniuchiTakehiko IgarashiMasataka KudoHideo HarigaeHiroshi FujiiPublished in: Modern rheumatology case reports (2020)
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.