CASE REPORTSSevere bleeding diathesis as onset of light-chain amyloidosis: combined excessive fibrinolysis and acquired von Willebrand disease in a young patientPardos-Gea, Joséa; Cortina, Vicenteb; Marques-Soares, Joana Ritaa; Martínez, Fernandab; Gironella, Mercheb Author Information aAutoimmune Diseases Unit, Department of Internal Medicine bDepartment of Hematology and Vall d’Hebrón Institute of Oncology (VHIO), Vall d’Hebrón University Hospital, Barcelona, Spain Correspondence to José Pardos-Gea, MD, PhD, Autoimmune Diseases Unit, Department of Internal Medicine, Vall d’Hebrón University Hospital, Passeig de la Vall Hebrón 115-119, 08035 Barcelona, Spain. E-mail: [email protected] Received 7 June, 2022 Accepted 24 July, 2022 Blood Coagulation & Fibrinolysis 34(1):p 79-81, January 2023. | DOI: 10.1097/MBC.0000000000001163 Buy Metrics 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. Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.