In the case of a 69-year-old man suffering from recurrent high fever, laboratory data demonstrated elevated inflammatory findings such as neutrophilic leukocytosis and an increase in serum level of C-reactive protein. A nonsteroidal antiinflammatory drug or a low dose of prednisolone proved to be temporarily effective.A bone marrow specimen showed myelodysplastic syndrome (MDS) of the refractory anemia (RA) type with trisomy 8. Seven months after onset, painful erythematous eruptions were found on both legs. Biopsy of a skin lesion demonstrated neutrophic infiltration into the dermis. As a result, Sweet’s syndrome (SS) was diagnosed on the basis of clinical and histopathological findings. Administration of prednisolone (30 mg/d) was started and resulted in prompt defervescence and resolution of the lesions within 1 week.We also investigated serum levels of 3 cytokines: interleukin 1β (IL-1β), IL-6, and granulocyte colony-stimulating factor (G-CSF). Levels of IL-6 and G-CSF were elevated during the active phase, but the level of IL-1β did not increase. Because cases of MDS with trisomy 8 and SS or Behçet disease have been reported recently, our findings suggested that cytokine production, which enhances neutrophil function, is elevated in some MDS patients with trisomy 8; that is, not only cytokines but also trisomy 8 may be related to the pathogenesis of SS in MDS. It is thus advisable to watch for development of SS during follow-up of MDS patients with recurrent fever or trisomy 8.
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Hattori, H., Hoshida, S. & Yoneda, S. Sweet’s Syndrome Associated with Recurrent Fever in a Patient with Trisomy 8 Myelodysplastic Syndrome. Int J Hematol 77, 383–386 (2003). https://doi.org/10.1007/BF02982648
- Behçet disease
- Myelodysplastic syndrome
- Recurrent fever
- Sweet’s syndrome
- Trisomy 8