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DIAGNOSTIC CHALLENGES IN ACQUIRED VON WILLEBRAND DISEASE: A COMPLEX CASE OF PROSTATE CARCINOMA
Author(s): ,
Paul-Emile Claus
Affiliations:
Clinical Biology,AZ Delta,Roeselare,Belgium
,
Els Moreau
Affiliations:
Clinical Biology,AZ Delta,Roeselare,Belgium
Dries Deeren
Affiliations:
Hematology,AZ Delta,Roeselare,Belgium
(Abstract release date: 05/19/16) EHA Library. Claus P. 06/09/16; 134655; PB1755
Dr. Paul-Emile Claus
Dr. Paul-Emile Claus
Contributions
Abstract
Abstract: PB1755

Type: Publication Only

Background
Acquired von Willebrand disease (AVWD) is a rare bleeding disorder associated with a variety of underlying diseases and with clinical manifestations similar to congenital von Willebrand disease.

Aims
We present a case of prostate carcinoma in which AVWD was diagnosed.

Methods
The patient was an 80-year-old man who suffered from ulcerative colitis. He was diagnosed with a Gleason 7 prostate adenocarcinoma for which he received radiotherapy in combination with hormonal therapy. Due to melena he underwent a coloscopy during which biopsies were taken. The procedure was complicated by diffuse bleeding and a cardiac infarction. Urgent coronary artery bypass surgery (CABG) was performed but was also complicated by postoperative bleeding. Laboratory studies showed a normal platelet count, a prolongation of the activated partial thromboplastin time (aPTT) and low factor VIII levels. His von Willebrand factor (VWF) antigen level and ristocetin cofactor activity were low.

Results
The diagnosis of AVWD in this patient challenged us to look for the different pathogenic mechanisms of the disease. Several disorders have been described to be associated with AVWD, the commonest being hematoproliferative, cardiovascular and autoimmune disorders. Neoplasms are rarely documented and their pathogenic mechanisms are not well known. The underlying mechanisms differ among these disorders and may even overlap. These include, among other things, mechanical destruction of VWF under high shear stress and development of autoantibodies. Selective and/or non-selective absorption of VWF on or inside malignant cells is believed to be the main mechanism in hematoproliferative and non-hematologic malignancies.

Conclusion
In this report we give a concise overview of the mechanisms of the diseases that we encountered in this complex case.

Session topic: E-poster

Keyword(s): Acquired von Willebrand syndrome, Cancer, Diagnosis, Prostate
Abstract: PB1755

Type: Publication Only

Background
Acquired von Willebrand disease (AVWD) is a rare bleeding disorder associated with a variety of underlying diseases and with clinical manifestations similar to congenital von Willebrand disease.

Aims
We present a case of prostate carcinoma in which AVWD was diagnosed.

Methods
The patient was an 80-year-old man who suffered from ulcerative colitis. He was diagnosed with a Gleason 7 prostate adenocarcinoma for which he received radiotherapy in combination with hormonal therapy. Due to melena he underwent a coloscopy during which biopsies were taken. The procedure was complicated by diffuse bleeding and a cardiac infarction. Urgent coronary artery bypass surgery (CABG) was performed but was also complicated by postoperative bleeding. Laboratory studies showed a normal platelet count, a prolongation of the activated partial thromboplastin time (aPTT) and low factor VIII levels. His von Willebrand factor (VWF) antigen level and ristocetin cofactor activity were low.

Results
The diagnosis of AVWD in this patient challenged us to look for the different pathogenic mechanisms of the disease. Several disorders have been described to be associated with AVWD, the commonest being hematoproliferative, cardiovascular and autoimmune disorders. Neoplasms are rarely documented and their pathogenic mechanisms are not well known. The underlying mechanisms differ among these disorders and may even overlap. These include, among other things, mechanical destruction of VWF under high shear stress and development of autoantibodies. Selective and/or non-selective absorption of VWF on or inside malignant cells is believed to be the main mechanism in hematoproliferative and non-hematologic malignancies.

Conclusion
In this report we give a concise overview of the mechanisms of the diseases that we encountered in this complex case.

Session topic: E-poster

Keyword(s): Acquired von Willebrand syndrome, Cancer, Diagnosis, Prostate

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