Henry Ford Hospital Medical Journal


A 65-year-old woman who had a documented history of dermatomyositis was hospitalized for evaluation of a syncopal attack. During the course of her stay, she experienced progressive neurologic dysfunction, hemolytic anemia, and thrombocytopenia. A clinical diagnosis of thrombotic thrombocytopenia purpura (TTP) was made, and therapy was initiated with glucocorticoids, plasmapheresis, plasma infusion, and antiplatelet medication. The pathogenesis of TTP has not been cleady elucidated. However, reports in the literature have postulated immune damage of the endothelium with demonstration of IgM and complement deposits as the origin of the condition. Further, there are reported cases of TTP associated with collagen disorders such as systemic lupus erythematosus, rheumatoid arthritis, polyarteritis nodosa, and Sjogren's syndrome. To our knowledge, this represents the first known case of an association of TTP with dermatomyositis. Because of the implication of an immunologic pathogenesis for these disorders, this association is worth noting.



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