Salvage therapy with high dose Intravenous Immunoglobulins in acquired Von Willebrand Syndrome and unresponsive severe intestinal bleeding

Massimo Cugno, Alberto Tedeschi, Simona M. Siboni, Francesca Stufano, Federica Depetri, Franca Franchi, Samantha Griffini, Flora Peyvandi

Research output: Contribution to journalArticle

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.

Original languageEnglish
Article number15
JournalExperimental Hematology and Oncology
Volume3
Issue number1
DOIs
Publication statusPublished - Jun 4 2014

Keywords

  • Acquired von Willebrand syndrome
  • Gastrointestinal bleeding
  • Intravenous immunoglobulin

ASJC Scopus subject areas

  • Hematology
  • Oncology
  • Cancer Research

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