Case of hemophagocytic syndrome associated with active dermatomyositis]

We report a case of dermatomyositis (DM) with hemophagocytic syndrome (HPS). The patient is a 60 year old male admitted to our hospital with muscle weakness, high fever, weight loss and pancytopenia. On physical examination, proximal muscle weakness and skin rash on the back were noted. Laboratory data revealed elevated serum levels of muscle enzymes, lactate dehydrogenase and ferritin. Serum levels of M-CSF, TNF-alpha, soluble IL 2 receptor were remarkably increased. Bone marrow aspiration showed histiocytosis of 7 to 10% with prominent hemophagocytosis. Gastroendoscopic examination revealed II-a gastric cancer. He was treated with methylprednisolone (m-PSL) pulse theraphy (1 g/ day x 3 days) followed by 60 mg/day oral prednisolone. He quickly responded to the treatment and laboratory data returned to normal in 20 days despite the remaining gastric cancer which was removed successfully 3 months later. HPS is a rare complication of DM and only three cases have been documented so far in the literature. Augmented cytokine in his serum is considered to be closely related to HPS in this case.