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A case of autoimmune severe acquired von Willebrand syndrome (type 3-like)
- Source :
- Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis. 56(3)
- Publication Year :
- 2017
-
Abstract
- Von Willebrand disease (VWD) is the most common congenital bleeding disorder and is due to quantitative or qualitative defects of von Willebrand factor (VWF). Acquired defects of VWF, termed acquired von Willebrand syndrome (AVWS), are due to a host of different mechanisms. Autoantibody-mediated AVWS may be associated with lymphoproliferative or immunological disorders, such as systemic lupus erythematosus (SLE). A large majority of AVWS cases are type 1 or type 2A-like and patients tend to have a mild to moderate bleeding tendency. We report a case of severe autoimmune AVWS in a woman with SLE who presented with clinical and laboratory features of type 3 VWD (undetectable VWF antigen, ristocetin cofactor activity, and VWF multimers). A mixing study demonstrated an inhibitor to VWF (6BU/mL). Her bleeds were managed with antifibrinolytics, recombinant activated factor VII, and activated prothrombin complex concentrate. She was initially treated with steroids and intravenous immunoglobulin therapy. However, her bleeding symptoms continued until she was treated with rituximab, and her VWF parameters normalized. She relapsed two years later due to non-compliance with her immunosuppressive medications and expired another two years later secondary to complications of sepsis and uremic pericarditis. This case emphasizes the importance of aggressive initial therapy of SLE to reduce secondary complications, frequent patient monitoring, and continued treatment of the underlying autoimmune disorder in patients with AVWS.
- Subjects :
- Adult
congenital, hereditary, and neonatal diseases and abnormalities
030204 cardiovascular system & hematology
Autoimmune Diseases
Sepsis
03 medical and health sciences
Young Adult
0302 clinical medicine
Intravenous Immunoglobulin Therapy
Von Willebrand factor
hemic and lymphatic diseases
Von Willebrand disease
Medicine
Humans
Congenital Bleeding Disorder
biology
business.industry
Hematology
Mixing study
medicine.disease
Survival Analysis
von Willebrand Diseases
030220 oncology & carcinogenesis
Immunology
biology.protein
Rituximab
Female
Uremic pericarditis
business
circulatory and respiratory physiology
medicine.drug
Subjects
Details
- ISSN :
- 14730502
- Volume :
- 56
- Issue :
- 3
- Database :
- OpenAIRE
- Journal :
- Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis
- Accession number :
- edsair.doi.dedup.....6052fa6d538244069706244819b3c099