Effective Management of Refractory Paraneoplastic Vasculitis in Myelodysplastic Syndromes With Azacitidine, Prednisolone, and Azathioprine

TL;DR

A 62-year-old man with a rare blood disorder and vasculitis was successfully treated with a combination of drugs, though his condition later worsened.

Contribution

This case demonstrates a successful treatment approach for refractory paraneoplastic vasculitis in MDS using azacitidine, prednisolone, and azathioprine.

Findings

The combination therapy effectively controlled vasculitis for 10 treatment courses.

The disease later transformed into acute myeloid leukemia despite initial success.

The case emphasizes the need for monitoring leukemic progression under prolonged immunosuppression.

Abstract

A 62-year-old man presented with left ear pain, sensorineural hearing loss, and high-grade fever. Peripheral blood tests revealed abnormal blood cells, prompting further investigation that led to a diagnosis of myelodysplastic syndromes (MDS) with paraneoplastic vasculitis. Since initial treatment with azacitidine (Aza) was insufficient, additional immunosuppressive therapy was required. The disease was effectively controlled with a combination of Aza, prednisolone, and azathioprine, with no relapse for 10 treatment courses. However, the disease later transformed into acute myeloid leukemia. This case highlights the efficacy and feasibility of additional treatment with steroids and azathioprine for refractory paraneoplastic vasculitis associated with MDS, while emphasizing the need for careful monitoring of leukemic progression under prolonged immunosuppression.

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