MYELODYSPLASTIC SYNDROME WITH VASCULITIC MANIFESTATIONS

Vasculitis in patients with the myelodysplastic syndrome (MDS) is a ra re phenomenon. We describe a patient who presented with necrotic lesio ns of his toes, which proved to be the result of immune complex mediat ed vasculitis. This unusual combination of vasculitis and MDS prompted us to review the literature. Forty-four cases of vasculitis in associ ation with MDS were found. The pathogenesis of the vasculitis in MDS r emains speculative, although several reports suggest an immunological mechanism. The temporal relationship could not be determined in 20 (45 %) of reported cases. The prognosis of these patients appears to be wo rse than in patients with MDS without vasculitis. Steroids were used i n 41 (93%) of the reported cases. Our patient did not receive any drug therapy, nevertheless his necrotic lesions improved within a few week s.