Systemic AL amyloidosis with disseminated intravascular coagulation associated with hyperfibrinolysis

Many coagulation abnormalities are known to coexist in patients with AL amyloidosis; however, disseminated intravascular coagulation (DIC) is rarely observed. We describe the case of a 61-year-old woman who presented with systemic purpura, macroscopic hematuria, and hepatosplenomegaly as the initial manifestations of systemic AL amyloidosis. A coagulation study revealed severe DIC associated with fibrinolysis. The patient was treated for DIC with gabexate mesilate (GM); however, her bleeding symptoms and thrombocytopenia continued to worsen. The treatment was changed from GM to nafamostat mesilate (NM); DIC improved gradually, and the platelet count normalized in 1 week. After the tapering and cessation of NM therapy, deterioration of DIC did not occur. She underwent autologous peripheral blood stem cell transplantation twice following high-dose melphalan therapy, and received maintenance therapy with thalidomide. Hepatosplenomegaly progression appears to have been halted, and DIC has not recurred. This is the first reported case of AL amyloidosis showing severe DIC with excessive fibrinolysis. The clinical observation that NM was considerably more effective than GM in our patient suggests that NM may be more suitable for the treatment of DIC with a hyperfibrinolytic condition in AL amyloidosis patients.