Acquired von Willebrand disease in a patient with Wilms tumor

Noronha, Peter A.; Hruby, Marilyn A.; Maurer, Helen S.

doi:10.1016/s0022-3476(79)80293-0

Cited by 55 publications

(33 citation statements)

References 7 publications

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“…In previous reports of acquired von Willebrand disease associated with nephroblastoma, the serum hyaluronic acid level was not evaluated [7,8]. Our studies indicate that hyaluronic acid interacts with F.VIUR: Ag rendering it undetectable by immunoelectrophoretic methods.…”

Section: Discussioncontrasting

confidence: 56%

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Platelet dysfunction associated with wilms tumor and hyaluronic acid

Bracey

Aceves

et al. 1987

American J Hematol

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Acquired von Willebrand disease (AVWD) has been described in two cases of nephroblastoma. We studied a patient with nephroblastoma who presented with a coagulopathy suggestive of AVWD. The subject had undetectable levels of F.VIIIR:Ag, diminished F.VIIIR:WF (16.3%), F.VIII:C activity (37%), and lack of platelet aggregation to ADP, epinephrine, collagen, and arachidonic acid. These results were associated with abnormally high serum levels (850 mg/dl) of hyaluronic acid (HA), which made the patient's serum hyperviscous. Examination of the neoplasm revealed HA in the tumor matrix. All abnormalities of coagulation resolved after chemotherapy and extirpation of the neoplasm, which produced normal serum HA levels. To study the effects of HA on platelet function, we added HA to normal platelet-poor plasma (NPP), which rendered F.VIIIR:Ag undetectable; treatment of HA with hyaluronidase eliminated F.VIIIR:Ag assay interference caused by HA. F.VIII:C activity decreased in vitro when HA was mixed with normal platelet-poor plasma (NPP). HA reduced the initial slope of normal platelet aggregation. Partial correction of platelet aggregation occurred after hyaluronidase treatment of HA-spiked PRP. Experiments in rabbits exposed to HA (serum level 400 mg/dl) demonstrated abnormalities similar to those noted in the patient. Shear rate studies of whole blood containing HA (500 mg/dl) yielded high shear stress, 27-136 dynes/cm2 over shear rates of 10-216 sec-1. We conclude that the coagulopathy demonstrated in this case is secondary to hyperviscosity produced by elevated levels of HA, which interferes with the assay for F.VIIIR:Ag. Thus the acquired coagulopathy associated with other cases of nephroblastoma may present as spurious von Willebrand disease.

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Section: Discussioncontrasting

confidence: 56%

“…In some reports, evidence of an immunoglobulin inhibitor of von Willebrand factor (F.VIIIR:WF) has been documented [3-51. AVWD has also been reported in other disorders including nephroblastoma [6,7].…”

Section: Introductionmentioning

confidence: 98%

Platelet dysfunction associated with wilms tumor and hyaluronic acid

Bracey

Aceves

et al. 1987

American J Hematol

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“…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]. Our study gives an incidence of 2/65, although this may represent an underestimate, as factor assays were not carried out in 7/16 patients with abnormal clotting.…”

Section: Discussionmentioning

confidence: 72%

Coagulopathy as a presenting feature of Wilms tumour

2004

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“…Because these patients often require resection of the tumor, treatment is frequently required to prevent surgical bleeding. [15][16][17] Although based on a very small number of treated patients, published reports describe use of DDAVP and replacement of VWF. However, the half-life of VWF is decreased because the infused factor also adsorbs to the tumor.…”

Section: Treatment Of Avws Caused By Adsorption To Malignancymentioning

confidence: 99%

Treatment of acquired von Willebrand syndrome in childhood

Callaghan

Wong

Federici

2013

Blood

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A 3-1/2-year-old male with no personal or family history of bleeding disorders presented with abdominal distension, epistaxis, and anemia (hemoglobin 8.2 g/dL). A magnetic resonance imaging scan of the abdomen demonstrated a mass arising from the left kidney. Preoperative laboratory studies revealed a prolonged activated partial thromboplastin time of 49.2 seconds, a normal prothrombin time of 12.4 seconds, and a platelet count of 230 000/mL. Further testing revealed factor VIII (FVIII) activity of 16%, factor IX (FIX) activity of 74%, von Willebrand factor (VWF) activity of 12%, VWF antigen activity of 31%, and decreased high-molecular-weight VWF multimers consistent with acquired von

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Acquired von Willebrand disease in a patient with Wilms tumor

Cited by 55 publications

References 7 publications

Platelet dysfunction associated with wilms tumor and hyaluronic acid

Platelet dysfunction associated with wilms tumor and hyaluronic acid

Coagulopathy as a presenting feature of Wilms tumour

Treatment of acquired von Willebrand syndrome in childhood

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