Hemophagocytic syndrome and metastatic melanoma: 3 cases

Background. Macrophage activation syndrome was initially described during v iral infections in immunocompromised patients. Since the original report, m any diseases have been found to be associated with macrophage activation sy ndrome. Lymphoproliferative disorders have been more frequently reported to be associated with macrophage activation syndrome than solid tumors. We he rein report three cases of macrophage activation syndrome in patients with metastatic malignant melanoma. Case-reports. Two young 32 and 40 year-old men with a liver metastatic mali gnant melanoma and a 62 year-old woman with a polymetastatic malignant mela noma presented a sudden deterioration of general health with hyperthermia a nd biological abnormalities: liver cytolysis, leucocytosis, thrombocytopeni a, hypertriglyceridaemia. A fatal clinical outcome occurred rapidly despite corticotherapy and/or chemotherapy. For the first two patients the macroph age activation syndrome diagnosis was delayed because of the similarities o f macrophage activation syndrome and metastatic malignant melanoma symptoms . Discussion. The diagnosis of macrophage activation syndrome in patients wit h metastatic malignant melanoma may be difficult because of the similaritie s between clinical features of macrophage activation syndrome and those of metastatic malignant melanoma. Hypertriglyceridaemia is present in 60 p. 10 0 of macrophage activation syndrome and should lead to process a bone marro w aspirate. The search for a triggering infection should be systematically carry out because it is implicated in more than half of macrophage activati on syndrome whatever the associated disease may be: neoplasia, autoimmune d isease... The pathogenesis of macrophage activation syndromes occurring in patients with metastatic cancer remains unexplained. Treatment of macrophag e activation syndrome is not unanimously established and usually consists i n the treatment of the associated condition as well as a corticosteroid and /or an immunosuppressive treatment regimens. Prognosis of macrophage activa tion syndrome is usually poor especially when it is associated with a neopl asia since a fatal outcome occurs in 40 to 60 p. 100 of cases.