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.
Salvage therapy with high dose Intravenous Immunoglobulins in acquired Von Willebrand Syndrome and unresponsive severe intestinal bleeding / M. Cugno, A. Tedeschi, S.M. Siboni, F. Stufano, F. Depetri, F.M. Franchi, S. Griffini, F. Peyvandi. - In: EXPERIMENTAL HEMATOLOGY & ONCOLOGY. - ISSN 2162-3619. - 3:1(2014), pp. 15.1-15.6.
|Titolo:||Salvage therapy with high dose Intravenous Immunoglobulins in acquired Von Willebrand Syndrome and unresponsive severe intestinal bleeding|
CUGNO, MASSIMO (Primo)
GRIFFINI, SAMANTHA (Penultimo)
PAYVANDI, FLORA (Ultimo)
|Parole Chiave:||Acquired von Willebrand syndrome; gastrointestinal bleeding; intravenous immunoglobulin|
|Settore Scientifico Disciplinare:||Settore MED/09 - Medicina Interna|
|Data di pubblicazione:||2014|
|Digital Object Identifier (DOI):||http://dx.doi.org/10.1186/2162-3619-3-15|
|Appare nelle tipologie:||01 - Articolo su periodico|