• N Cordel, C Le Corvaisier-Piéto, P Young, B Lenormand, P Courville, J Soubrane, P Joly, and P Lauret.
• Clinique Dermatologique, CHU de Rouen.
• Ann Dermatol Vener. 2000 Dec 1;127(12):1077-9.

BackgroundMacrophage activation syndrome was initially described during viral infections in immunocompromised patients. Since the original report, many diseases have been found to be associated with macrophage activation syndrome. Lymphoproliferative disorders have been more frequently reported to be associated with macrophage activation syndrome than solid tumors. We herein report three cases of macrophage activation syndrome in patients with metastatic malignant melanoma.Case ReportsTwo young 32 and 40 year-old men with a liver metastatic malignant melanoma and a 62 year-old woman with a polymetastatic malignant melanoma presented a sudden deterioration of general health with hyperthermia and biological abnormalities: liver cytolysis, leucocytosis, thrombocytopenia, hypertriglyceridaemia. A fatal clinical outcome occurred rapidly despite corticotherapy and/or chemotherapy. For the first two patients the macrophage activation syndrome diagnosis was delayed because of the similarities of macrophage activation syndrome and metastatic malignant melanoma symptoms.DiscussionThe diagnosis of macrophage activation syndrome in patients with metastatic malignant melanoma may be difficult because of the similarities between clinical features of macrophage activation syndrome and those of metastatic malignant melanoma. Hypertriglyceridaemia is present in 60 p. 100 of macrophage activation syndrome and should lead to process a bone marrow aspirate. The search for a triggering infection should be systematically carry out because it is implicated in more than half of macrophage activation syndrome whatever the associated disease may be: neoplasia, autoimmune disease. The pathogenesis of macrophage activation syndromes occurring in patients with metastatic cancer remains unexplained. Treatment of macrophage activation syndrome is not unanimously established and usually consists in the treatment of the associated condition as well as a corticosteroid and/or an immunosuppressive treatment regimens. Prognosis of macrophage activation syndrome is usually poor especially when it is associated with a neoplasia since a fatal outcome occurs in 40 to 60 p. 100 of cases.

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