10 September 2020>: Artilces
Autoimmune Myelofibrosis in Sjögren’s Syndrome: Report of a Case
Rare disease
Seiji Kakiuchi A* , Ikumi Takagi A , Hiroaki Akiyama B , Hiroyuki Matsuba B , Junpei Rikitake B , Kazuyoshi Kajimoto B , Yoshitake Hayashi B , Nobuko Iwata BDOI: 10.12659/AJCR.924983
Am J Case Rep 2020; 21:e924983
Table 1. Previous reports of autoimmune myelofibrosis accompanied by Sjögren’s syndrome.
Age/Sex | Initial therapy | Hematological response | Bone marrow response | Recurrence | Maintenance therapy | |
---|---|---|---|---|---|---|
1. Gruson et al. []7 | 30/F | PSL 1 mg/kg/day for 1 month→ tapered off over next 3 months | (+) | ND | (+) at 5 months | Normalized within 2 weeks after azathioprine administration |
2. Hattori et al. | 72/F | PSL 1 mg/kg/day (50 mg/body)→ tapered to 10 mg in just over 2 months | (+) 1 month | (+) | (–) | PSL 4 mg/day at 4 months |
3. Marie et al. []8 | 59/F | mPSL 500 mg×3 days→ PSL 1 mg/kg/day (70 mg/body) | (+) 1 month | ND | (–) | PSL 15 mg/day at 12 months |
4. Rizzi et al. []9 | 43/M | PSL 1 mg/kg/day→ tapered off over next 6 months | (+) | (–) | (+) | Restarted lowest effective dose of PSL |
5. Rizzi et al. []9 | 66/F | PSL 4 mg/day+CsA 50 mg/day for 4 days every week+hydroxychloroquine 200 mg/day for 12 months | (–) | ND | ND | Hydroxychloroquine+low dose PSL |
CsA – cyclosporine A; F – Female; M – Male; mPSL – methylprednisolone; ND – not described; PSL – prednisolone; WBC – white blood count. |