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Differential diagnostic and treatment difficulties in a patient with acquired von Willebrand syndrome
- Source :
- Hematology. 26:301-304
- Publication Year :
- 2021
- Publisher :
- Informa UK Limited, 2021.
-
Abstract
- Acquired von Willebrand syndrome (AVWS) is a rare, frequently underdiagnosed and underestimated bleeding disorder. Careful personal and family history and late-onset mucocutaneous bleeding could help clarify the etiology of bleeding deficiency.An 82-year-old male patient was admitted to our clinic with a severe nosebleed on 30.05.2018. Laboratory results revealed thrombocytosis, elevated white blood cell count and high LDH. Basic coagulation parameters were normal. He was referred to our clinic, where a bone marrow biopsy was taken. His personal and family history had no mention of bleeding disorders, nor was he on anticoagulant therapy. We detected elevated VWF antigen and decreased VWF ristocetin cofactor activity. Loss of high molecular weight multimers was detected by using agarose gel electrophoresis. These laboratory results were indicative of AVWS. Hydroxyurea treatment was initiated, leading to a gradual decrease in platelet count. The histological examination revealed essential thrombocytosist while mutation analysis was JAK2/CALR/MPL negative. However, due to severe nosebleeds, the patient was hospitalized and needed blood transfusion. A cardiological check-up revealed the progression of aortic valve stenosis. After, balloon-dilation a transcatheter aortic valve implantation was performed. As a result, VWF activity and activity to antigen ratio returned to normal as did multimeric structure. In July 2019, the follow-up examination showed that the patient was in a satisfactory condition, with normal hematological parameters, and no new nosebleed episode occurred.The patient complained of recurring nosebleeds, which stopped completely after the resolution of both underlying conditions successful cytoreductive treatment of triple-negative ET and transcatheteric aortic valve replacement.
- Subjects :
- Male
medicine.medical_specialty
Mucocutaneous bleeding
Hemorrhage
03 medical and health sciences
0302 clinical medicine
Acquired von Willebrand syndrome
von Willebrand Factor
medicine
Humans
Hydroxyurea
Blood Transfusion
Enzyme Inhibitors
Family history
Myeloproliferative neoplasm
Aged, 80 and over
Thrombocytosis
Essential thrombocythemia
business.industry
Hematology
medicine.disease
Dermatology
von Willebrand Diseases
030220 oncology & carcinogenesis
business
030215 immunology
Subjects
Details
- ISSN :
- 16078454
- Volume :
- 26
- Database :
- OpenAIRE
- Journal :
- Hematology
- Accession number :
- edsair.doi.dedup.....bb1204900dc747af36ecbed08dfc8c12