Salvage therapy with high dose Intravenous Immunoglobulins in acquired Von Willebrand Syndrome and unresponsive severe intestinal bleeding
| Autor: | Flora Peyvandi, Federica Depetri, Simona Maria Siboni, Franca Franchi, Alberto Tedeschi, Francesca Stufano, Massimo Cugno, Samantha Griffini |
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| Rok vydání: | 2014 |
| Předmět: |
Cancer Research
medicine.medical_specialty Gastrointestinal bleeding Salvage therapy Case Report Acquired von Willebrand syndrome Gastroenterology Antigen Internal medicine Medicine Intravenous immunoglobulin Hematology biology business.industry Autoantibody medicine.disease Surgery Red blood cell medicine.anatomical_structure Oncology Immunoglobulin M biology.protein Hemoglobin business |
| Zdroj: | Experimental Hematology & Oncology |
| ISSN: | 2162-3619 |
| DOI: | 10.1186/2162-3619-3-15 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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