TITLE

Atypical Variant of Acquired von Willebrand Syndrome in Wilms Tumor: Is Hyaluronic Acid Secreted by Nephroblastoma Cells the Cause?

AUTHOR(S)
Michiels, Jan Jacques; Schroyens, Wilfried; Berneman, Zwi; van der Planken, Marc
PUB. DATE
April 2001
SOURCE
Clinical & Applied Thrombosis/Hemostasis;Apr2001, Vol. 7 Issue 2, p102
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
ABSTRACT
Acquired von Willebrand syndrome (AvWS) has been reported in eight children with Wilms lumor (nephroblaslonia in four boys and four girls) at a mean age of 3.3 years (range. 0.33–9 years). Only three of eight patients with AvWS in Wilms tumor presented with mild mucocutaneous bleeding symptoms. The AvWS in seven children with Wilms tumor featured either undeteclable or very low von Willebrand factor antigen (vWF.Ag) levels (mean. 3%) and decreased values for vWF ristocetin cofactor (RCF) activity (mean, 20%) and factor VIII coagulant (VIIIc) activity (mean. 16%). The response to l-desamino-8-arginine vasopressin (DDAVP) was good in two and poor in one patient. Multimeric analysis of the vWF showed a normal pattern of type 1 von Willebrand disease (vWD) in three patients and an absence of multimers consistent with type III vWD in two patients. The higher functional levels. as compared with antigen levels, with increased ratios for factor VIIIc/vWFAg (mean, 5.3) and vWF.RCF/vWF.Ag (mean. 6.6) in seven patients with Wilms tumor are unexplained physiologically and are not consistent with type I vWF deficiency. The absence of vWD in the patient's family, and the return of factor VIII-vWF parameters to normal after chemotherapy or surgical removal of the Wilms tumor, support the diagnosis of AvWS causally related to the Wilms tumor. The causative agent is thought to be hyaluronic acid secreted by nephroblastoma cells of the Wilms tumor. Prospective studies to determine the nature of AvWS in children with Wilms tumor are warranted.
ACCESSION #
22385468

 

Related Articles

  • Considerations for epidural anesthesia in a patient with type 1 von Willebrand disease. HARA, KOJI; KISHI, NOBUYUKI; SATA, TAKEYOSHI // Journal of Anesthesia;2009, Vol. 23 Issue 4, p597 

    Epidural anesthesia, combined with general anesthesia, was performed in a patient with type 1 von Willebrand disease (VWD). VWD is a common inherited bleeding disorder, and an anesthetic concern in patients with VWD is susceptibility to postoperative bleeding and epidural or spinal hematoma...

  • Coagulopathy as a presenting feature of Wilms tumour. Leung, Rebecca S.; Liesner, Ri; Brock, Penelope // European Journal of Pediatrics;2004, Vol. 163 Issue 7, p369 

    Unlabelled: Wilms' tumour (nephroblastoma) is the most common intrarenal malignancy of childhood. The most common presentation is of an asymptomatic abdominal mass (more than 80%), usually discovered incidentally. Abdominal pain occurs in 30% to 40% of patients, and hypertension,...

  • von Willebrand Disease Laboratory Diagnosis. Marques, Marisa B.; Fritsma, George A. // American Journal of Clinical Pathology;Jun2011, Vol. 135 Issue 6, p818 

    The article offers clinical information on the autosomal disorder von Willebrand disease (vWD). The prevalence of vWD, which is characterized by episodic mucocutaneous bleeding, is estimated at a range of 0.01% to 1.0% depending on the definition and population basis. Common symptoms of the...

  • Do Hemophilia A and von Willebrand Disease Protect Against Carotid Atherosclerosis? A Comparative Study Between Coagulopathics and Normal Subjects by Means of Carotid Echo-Color Doppler Scan. Bilora, Franca; Dei Rossi, Camilla; Girolami, Bruno; Casonato, Alessandra; Zanon, Ezio; Bertomoro, Antonella; Girolami, Antonio // Clinical & Applied Thrombosis/Hemostasis;Oct1999, Vol. 5 Issue 4, p232 

    Atherosclerosis is a multifactorial disease caused by genetic and environmental factors with important clinical sequelae. The aim of this study was to evaluate the degree of carotid atherosclerosis by echo-color Doppler scan in a group of patients affected by hemophilia A and von Willebrand...

  • Situación de salud oral de niños uruguayos portadores de coagulopatías hereditarias. Centro Hospitalario Pereira Rossell, Montevideo, Uruguay. Hermida Bruno, María Laura; Álvarez Loureiro, Licet; Lewis, Willy; Gabriel, Lucía; Boggia Tutaglio, Beatriz; Segovia, Adriana; Rodríguez Grecco, Ismael // Universitas Odontológica;mar2011, Vol. 30 Issue 64, p31 

    Aim: Describe the oral health status of children with inherited bleeding disorders who attended the Pereira Rossell Hospital in Montevideo Uruguay for dental care, between February 2008 and December 2009, and compare it with children without bleeding disorders. Methods: A retrospective...

  • Clinical features and types of Von Willebrand disease in women with menorrhagia referred to Hematology Clinic of Kermanshah. Payandeh, Mehrdad; Rahimi, Zohreh; Kansestani, Atefeh Nasir; Hemmati, Shahrooz; Aleyasin, Mahnaz; Zare, Mohammad Erfan; Nouri, Zohreh; Hashemian, Amir Hossein; Gohardehi, Farzad // International Journal of Hematology-Oncology & Stem Cell Researc;Apr2013, Vol. 7 Issue 2, p1 

    Menorrhagia is the most common symptom that is experienced by women with bleeding disorders. Von Willebrand disease (VWD) is the most common congenital human bleeding disorder that is manifested as a quantitative deficiency in Von Willebrand factor (VWF) or dysfunction of this factor. The...

  • Co-existing mild Haemophilia A with Mild Type 1 Von Willebrand Disease: Case Report. AKIN, Mehmet; KARAPINAR, Deniz Yilmaz; BALKAN, Can; AY, Yilmaz; KAVAKLI, Kaan // International Journal of Hematology & Oncology / UHOD: Uluslarar;Jun2011, Vol. 21 Issue 2, p111 

    Von Willebrand disease and haemophilia A are the two most common inherited bleeding disorders. Despite the relatively high frequency of those two bleeding disorders in the general population, reports of their coexistence together or of combined coagulopathies in general are rare. We describe a...

  • Von Willebrand's disease.  // British Medical Journal;9/25/1976, Vol. 2 Issue 6038, p715 

    Investigates the factors of the deficiency of coagulation factor VIII and autosomal von Willebrand's disease. Response of factor VIII coagulation activity to transfusion; Evaluation of the response in von Willebrand's disease; Possibility of deficient synthesis of factor VIII related antigen in...

  • Disorders of coagulation in pregnancy. Katz, D.; Beilin, Y. // BJA: The British Journal of Anaesthesia;Dec2015 Supplement 2, Vol. 115, pii75 

    The process of haemostasis is complex and is further complicated in the parturient because of the physiological changes of pregnancy. Understanding these changes and the impact that they have on the safety profile of the anaesthetic options for labour and delivery is crucial to any anaesthetist...

Share

Read the Article

Courtesy of THE LIBRARY OF VIRGINIA

Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics