Pure red cell aplasia in autoimmune polyglandular syndrome with T lymphocytosis

We report the onset of pure red cell aplasia (PRCA) in a patient with a his tory of polyglandular syndrome including Addison's disease, malabsorption s yn; drome, diabetes type I and gastric hyperplastic polyposis. An autoimmun e origin for this complex disorder was not supported by the presence of org an specific antibodies, but T cells were found to be of polyclonal origin, as demonstrated by molecular analysis of T cell receptor (TCR) gene rearran gement. The pathophysiology of this case, based on laboratory findings and response to therapy, is discussed.