Systemic medium-sized vessel vasculitis associated with chronic myelomonocytic leukemia

Objective: To determine the clinical aspects of systemic vasculitis associa ted with chronic myelomonocytic leukemia (CMML). Methods: In this retrospective study, 8 patients suffering from systemic va sculitis associated with CMML are described. The French and English literat ure on systemic vasculitis associated with myelodysplasia was reviewed. Results: All 8 patients had a systemic medium-sized vessel vasculitis which fulfilled the American College of Rheumatology criteria for polyarteritis nodosa in the setting of active CMML. Antineutrophil cytoplasmic antibodies (ANCA) were negative in 7 patients. One patient had cytoplasmic ANCA by in direct immunofluorescence without antiproteinase 3 or antimyeloperoxydase a ntibodies on the enzyme-linked immunosorbent assay. At presentation, 6 pati ents had fever of unknown origin, 5 had polymyalgia rheumatica, 3 had senso ry hearing loss, and 4 had eosinophilia. None had viral infection or drug-a ssociated vasculitis. Diagnostic procedures included renal or hepatic angio graphy in 6 patients which showed microaneurysms in 4, skin and temporal ar tery biopsy in 2 which showed vasculitis, and 1 postmortem examination whic h showed gastroduodenal arteritis. All patients were treated with corticost eroids, and 7 received immunosuppressive drugs. Death was attributable to v asculitis in 2 cases, infection in 3, and other vasculitis-related causes i n 2. In a review of the French-English literature, we found 11 similar case s of ANCA-negative systemic vasculitis, generally associated with refractor y anemia, with or without blast excess. Conclusions: Systemic ANCA-negative polyarteritis nodosa-type vasculitis se ems closely associated to CMML. Clinical presentation is nonspecific, and s ystemic vasculitis should be suspected when a patient with myelodysplasia d evelops atypical manifestations. Renal, gastrointestinal, or hepatic angiog raphy are useful diagnostic procedures when more invasive biopsies should b e avoided because of low platelet count. The prognosis of CMML-associated s ystemic vasculitis is poor.