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Cugno M, Tedeschi A, Siboni SM, et al. Salvage therapy with high dose Intravenous Immunoglobulins in acquired Von Willebrand Syndrome and unresponsive severe intestinal bleeding. Exp Hematol Oncol. 2014;3:15doi: 10.1186/2162-3619-3-15.
Cugno, M., Tedeschi, A., Siboni, S. M., Stufano, F., Depetri, F., Franchi, F., Griffini, S., & Peyvandi, F. (2014). Salvage therapy with high dose Intravenous Immunoglobulins in acquired Von Willebrand Syndrome and unresponsive severe intestinal bleeding. Experimental hematology & oncology, 315. https://doi.org/10.1186/2162-3619-3-15
Cugno, Massimo, et al. "Salvage therapy with high dose Intravenous Immunoglobulins in acquired Von Willebrand Syndrome and unresponsive severe intestinal bleeding." Experimental hematology & oncology vol. 3 (2014): 15. doi: https://doi.org/10.1186/2162-3619-3-15
Cugno M, Tedeschi A, Siboni SM, Stufano F, Depetri F, Franchi F, Griffini S, Peyvandi F. Salvage therapy with high dose Intravenous Immunoglobulins in acquired Von Willebrand Syndrome and unresponsive severe intestinal bleeding. Exp Hematol Oncol. 2014 Jun 04;3:15. doi: 10.1186/2162-3619-3-15. eCollection 2014. PMID: 24926417; PMCID: PMC4055248.
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Exp Hematol Oncol. 2014 Jun 04;3:15. doi: 10.1186/2162-3619-3-15. eCollection 2014.

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

Experimental hematology & oncology

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

Affiliations

  1. Department of Pathophysiology and Transplantation, University of Milan, Milano, Italy ; Medicina Interna, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milano, Italy.
  2. Unità Operativa di Allergologia e Immunologia Clinica, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milano, Italy.
  3. 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.
  4. 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.

PMID: 24926417 PMCID: PMC4055248 DOI: 10.1186/2162-3619-3-15

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.

Keywords: Acquired von Willebrand syndrome; Gastrointestinal bleeding; Intravenous immunoglobulin

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