Severe bleeding diathesis as onset of light-chain amyloidosis: combined excessive fibrinolysis and acquired von Willebrand disease in a young patient

Abstract

Our report shows a case of primary light-chain amyloidosis in a young patient that reflects the potential severity of bleeding diathesis associated with this plasma cell dyscrasia and the difficulty of diagnosis when only hemorrhagic manifestations are present at the onset of disease. The patient presented with recurrent and severe muscular bleeding secondary to associated acquired von Willebrand disease and fibrinolysis dysfunction. Treatment with bortezomib-cyclophosphamide and sequential hematopoietic stem cell transplantation solved coagulation alterations. On the basis of our case, we review previous reports and discuss the potential mechanism of dysfunction of coagulation in light-chain amyloidosis.