Acquired von Willebrand disease in children with a Wilms' tumor

Poerink-Stockschläder, Anne B. Jonge; Dekker, I.; Risseeuw-Appel, I. M.; Haählen, Karel

doi:10.1002/(sici)1096-911x(199604)26:4<238::aid-mpo3>3.0.co;2-k

Cited by 34 publications

(18 citation statements)

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“…Before 1996 there had been five isolated case reports. Jonge Poerink-Stockschlader et al [8] carried out a 23-year retrospective study and found 2/73 with AVWS (2.7%). A further three cases have been reported [4,13,14].…”

Section: Discussionmentioning

confidence: 99%

“…Although in all reported cases in the literature the AVWS associated with Wilms' tumour resolved upon treating the tumour, it is imperative that the clotting be corrected perioperatively to reduce the risk of haemorrhage [3,4,8,13,14]. It has been demonstrated that the incidence of this disorder is perhaps higher than had previously been accepted [3] and therefore it is pertinent to be aware of its existence when presented with a newly diagnosed Wilms' tumour.…”

Section: Discussionmentioning

confidence: 99%

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Coagulopathy as a presenting feature of Wilms tumour

2004

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Coagulopathy as a presenting feature of Wilms tumour

2004

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“…Some centres advise coagulation studies because WT patients can develop acquired von Willebrand disease (17,18).…”

Section: Investigationmentioning

confidence: 99%

Management of Bilateral Wilms Tumours

2016

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Licence: This open access article is licensed under Creative Commons Attribution 4.0 International (CC BY 4.0). http://creativecommons.org/licenses/by/4.0/ Users are allowed to share (copy and redistribute the material in any medium or format) and adapt (remix, transform, and build upon the material for any purpose, even commercially), as long as the authors and the publisher are explicitly identified and properly acknowledged as the original source. AbstractSynchronous bilateral Wilms tumours (BWTs) represent 4% to 7% of all Wilms tumours (WTs) and present at a younger age than unilateral WTs do. At least 10% of synchronous BWTs have unfavourable histology, and up to 22% are associated with genitourinary abnormalities, aniridia, WAGR (WT, aniridia, genitourinary anomalies, and retardation) syndrome, Denys-Drash syndrome, hemihypertrophy or one of the other overgrowth syndromes. The long-term disease-free survival rate of patients with unilateral WT is now approaching 90% and is around 70% for those with metastatic disease. For both synchronous and metachronous WTs, the prognosis is less favourable, with reported cure rates 62approaching 80% in the best centres but are often considerably less in resource-poor settings. Also, there is the potential for a reduced quality of life due to renal insufficiency and the possible need for renal transplantation. Thus, the major clinical challenge in BWTs is the preservation of functioning renal tissue, while achieving cure with the minimum of therapyrelated morbidity. Although mortality is generally associated with progressive disease of anaplastic tumours, the emphasis of management has been increasingly placed on nephronsparing surgical approaches in an attempt to reduce ultimate renal insufficiency. Chemotherapy followed by nephron-sparing surgery has been able, in most cases, to eradicate the tumour while preserving renal function. Radiotherapy has largely been avoided because of fear of long-term radiation injury to the residual functioning renal mass. Patient selection, appropriate pre-and post-operative chemotherapy, and skilful surgical techniques all contribute to excellent outcomes, where these are achievable.

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“…Most cases of myeloproliferative disorders associated with aVWS are essential thrombocythemia [37][38][39][40][41][42][43][44][45][46][47], while polycythemia vera [48] and chronic myeloid leukemia [49][50][51] are less frequent. The second most common groups of disorders associated with aVWS is neoplasia, including Wilms tumors [52][53][54][55][56][57] and carcinomas [58,59], followed by immunological diseases, most notably SLE [14,60,61] and hypothyroidism [62][63][64][65][66][67][68][69][70][71][72][73][74][75]. Various other clinical conditions (Table I), such as rare immunological disorders [76][77][78][79], cardiovascular diseases [80][81]…”

Section: Associated Disorders and Pathogenic Mechanismsmentioning

confidence: 99%

Acquired von Willebrand syndrome: An update

Franchini

Lippi

2006

American J Hematol

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Acquired von Willebrand syndrome (aVWS) is a rare bleeding disorder with laboratory findings similar to those for congenital von Willebrand disease (VWD). However, unlike congenital VWD, it arises in individuals with no personal or family history of bleeding. aVWS occurs in association with a variety of underlying disorders, most frequently in lymphoproliferative disorders, myeloproliferative disorders, and cardiovascular diseases. Through an analysis of the more recent literature data, the pathophysiology and the clinical, laboratory, and therapeutic aspects of this syndrome are concisely reported in this review. Am. J. Hematol. 82:368-375, 2007. V V C 2006 Wiley-Liss, Inc.

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Acquired von Willebrand disease in children with a Wilms' tumor

Cited by 34 publications

References 20 publications

Coagulopathy as a presenting feature of Wilms tumour

Coagulopathy as a presenting feature of Wilms tumour

Management of Bilateral Wilms Tumours

Acquired von Willebrand syndrome: An update

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