title: Salvage therapy with high dose Intravenous Immunoglobulins in acquired Von Willebrand Syndrome and unresponsive severe intestinal bleeding.
abstract: A 91-year-old woman affected with acquired Von Willebrand (VW) syndrome and intestinal angiodysplasias presented with severe gastrointestinal bleeding (hemoglobin 5 g/dl). Despite replacement therapy with VW factor/factor VIII concentrate qid, bleeding did not stop (eleven packed red blood cell units were transfused over three days). High circulating levels of anti-VW factor immunoglobulin M were documented immunoenzimatically. Heart ultrasound showed abnormalities of the mitral and aortic valves with severe flow alterations. When intravenous immunoglobulins were added to therapy, prompt clinical and laboratory responses occurred: complete cessation of bleeding, raise in hemoglobin, VW factor antigen, VW ristocetin cofactor and factor VIII levels as well as progressive reduction of the anti-VWF autoantibody levels.
authors.affiliation: Department of Pathophysiology and Transplantation, University of Milan, Milano, Italy ; Medicina Interna, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milano, Italy.
authors.forename: Massimo
authors.initials: M
authors.lastname: Cugno
authors.affiliation: Unità Operativa di Allergologia e Immunologia Clinica, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milano, Italy.
authors.forename: Alberto
authors.initials: A
authors.lastname: Tedeschi
authors.affiliation: Department of Pathophysiology and Transplantation, University of Milan, Milano, Italy ; Angelo Bianchi Bonomi Haemophilia and Thrombosis Centre, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, and Luigi Villa Foundation, Milan, Italy.
authors.forename: Simona Maria
authors.initials: SM
authors.lastname: Siboni
authors.affiliation: Department of Pathophysiology and Transplantation, University of Milan, Milano, Italy ; Angelo Bianchi Bonomi Haemophilia and Thrombosis Centre, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, and Luigi Villa Foundation, Milan, Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: Stufano
authors.affiliation: Department of Pathophysiology and Transplantation, University of Milan, Milano, Italy ; Medicina Interna, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milano, Italy.
authors.forename: Federica
authors.initials: F
authors.lastname: Depetri
authors.affiliation: Department of Pathophysiology and Transplantation, University of Milan, Milano, Italy ; Angelo Bianchi Bonomi Haemophilia and Thrombosis Centre, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, and Luigi Villa Foundation, Milan, Italy.
authors.forename: Franca
authors.initials: F
authors.lastname: Franchi
authors.affiliation: Department of Pathophysiology and Transplantation, University of Milan, Milano, Italy ; Medicina Interna, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milano, Italy.
authors.forename: Samantha
authors.initials: S
authors.lastname: Griffini
authors.affiliation: Department of Pathophysiology and Transplantation, University of Milan, Milano, Italy ; Medicina Interna, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milano, Italy ; Angelo Bianchi Bonomi Haemophilia and Thrombosis Centre, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, and Luigi Villa Foundation, Milan, Italy.
authors.forename: Flora
authors.initials: F
authors.lastname: Peyvandi
keywords: Acquired von Willebrand syndrome; Gastrointestinal bleeding; Intravenous immunoglobulin