Autoimmune phenomena in patients with myelodysplastic syndromes

Autoimmune syndromes are common in patients with myelodysplastic syndromes (MDS). Clinical manifestations include an acute systemic vasculitic syndrome (characterized by skin vasculitis, fever, arthritis and sometimes associated with pulmonary infiltrates and peripheral edema), chronic autoimmune disorders, including chronic cutaneous vasculitis, polyneuropathy, inflammatory bowel disease and glomerulonephritis, and classical connective tissue disorders, most notably relapsing polychondritis. Asymptomatic immunologic abnormalities are also common and include hypergammaglobulinemia and a positive FANA. Autoimmune syndromes may be the primary cause of death in some patients with MDS. However, these syndromes frequently respond to immunosuppressive agents and occasional dramatic hematologic responses to steroid therapy are seen. We review the incidence, nature, course and response to therapy of these manifestations and discuss potential pathogenic mechanisms.