Acquired von willebrand disease: Correction of hemostatic defect by high‐dose intravenous immunoglobulins

Delmer, Alain; Horellou, Marie‐Hélène; Bréchot, J.-M.; Prudent, J; Potevin, F.; Lecrubier, C; Girard-Longhini, C; Samama, M; Zittoun, R

doi:10.1002/ajh.2830400214

Cited by 38 publications

(18 citation statements)

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“…Extracorporeal immunoadsorption was attempted in one patient with short-term success [53]. Consistent with our findings, there have been many reports of successful IvIg infusion for AvWD [17,[20][21][22]47,54]. The mechanism is unknown, but might be related to an effect of IvIg on the clearance of vWF-antibody complexes.…”

Section: Discussionsupporting

confidence: 91%

Idiopathic immune-mediated acquired von Willebrand's disease in a patient with angiodysplasia: Demonstration of an unusual inhibitor causing a functional defect and rapid clearance of von Willebrand factor

et al. 1999

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A case of idiopathic immune-mediated von Willebrand's disease (AvWD) associated angiodysplasia and recurrent lower gastrointestinal bleeding is reported. Coagulation parameters at presentation were activated partial thromboplastin time of 41 sec, bleeding time >15 min, factor VIII procoagulant activity, 5%; von Willebrand factor antigen (WF:Ag) 5%, and vWF:ristocetirn cofactor activity 11% sodium dodecyl sulfate-agarose gel electrophoresis pattern of plasma vWF showed a pattern similar to type II vWD. An in vitro inhibitor against vWF in the immunoglobulin (Ig)G fraction of the patient's plasma was demonstrated vWF parameters showed a short-lived increase after 1-deamino-8-D-arginine vasopressin (DDAVP) administration. The patient's bleeding episodes were initially managed adequately with cryoprecipitate replacement therapy and DDAVP, to which she became refractory. No significant improvement was achieved following the institution of immunosuppressive therapy in the form of high-dose steroids and cyclophosphamide. She was then treated with intravenous immunoglobulin (IvIg) to which she showed an adequate response in terms of her clinical situation and her hemostatic parameters. The patient is on maintenance treatment with repeated courses of IvIg based on vWF parameter monitoring. To our knowledge, this is the third reported association between idiopathic immune-mediated AvWD and angiodysplasia.

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Section: Discussionsupporting

confidence: 91%

Idiopathic immune-mediated acquired von Willebrand's disease in a patient with angiodysplasia: Demonstration of an unusual inhibitor causing a functional defect and rapid clearance of von Willebrand factor

et al. 1999

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“…More rigorous 10-20 days [19,20,22,23,27,30,42]. Castaman et al [21] found a short-lived effect of only 5 days after the initial investigation of AvWD in these situations is required.…”

Section: Table I Laboratory Values Of Patient Upon Admission Tomentioning

confidence: 99%

“…Discontinuation of drugs thought to cause AvWD apthe plasma was incubated with an anti-Fc IgG antibody prior to adding ristocetin. Since that study, there have been pears to result uniformly in resolution of the disorder, and persistent disease has not been reported in patients many reports of successful IVIG infusions for AvWD [13,19,[20][21][22][23]27,30,34,42]. In general, 0.5-2 g/kg IVIG with drug-induced AvWD [44][45][46][47].…”

Section: Table I Laboratory Values Of Patient Upon Admission Tomentioning

confidence: 99%

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Acquired von Willebrand's disease: A concise review

1997

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Acquired von Willebrand's disease (AvWD), an adult-onset bleeding diathesis, has most commonly been found in patients with an underlying lymphoproliferative disease or monoclonal gammopathy. Other malignancies, autoimmune diseases, hypothyroidism, and drugs have also been associated with AvWD. We have included an illustrative case history of a patient with a bleeding diathesis consistent with AvWD and a monoclonal gammopathy who required emergent cardiac surgery. Our review of the literature determined that most cases of AvWD are due to a circulating antibody that combines with the high molecular weight multimers ( CASE HISTORY INTRODUCTIONA 55-year-old man presented with severe epistaxis reAcquired von Willebrand's disease (AvWD) is an unquiring nasal packing and balloon tamponade. The patient common bleeding disorder that has remained, until recomplained of exertional chest pain for the past 2 weeks; cently, both difficult to characterize pathophysiologically, he now noted dyspnea and chest pain at rest. He was and challenging to treat successfully. Acquired forms of found to have an hematocrit of 22% and an elevated PTT, von Willebrand's disease are usually encountered in and he was transfused to a hematocrit of 30% with relief of adults without a personal or family history of bleeding his resting pain. Stress thallium showed diffuse, reversible diatheses [1]. Laboratory examination demonstrates that perfusion defects in the anterior, apical, inferior, and septhe functional assay for von Willebrand factor (vWF), tal areas, while an echocardiogram showed normal sysristocetin cofactor activity (vWF:RiCo), is characteristitolic function. The patient was referred to the University cally low or nearly absent, while the antigen (vWF:Ag) of Maryland Medical Center for cardiac catheterization assay is low or normal [2]. Bleeding time is markedly and evaluation of his bleeding disorder. prolonged. Furthermore, electrophoresis of von WilleThe patient's past medical history and family history brand factor most commonly reveals a pattern similar to type II congenital von Willebrand's disease (vWD), with selective loss of high molecular weight multimers *Correspondence to: Henry M. Rinder, M.D., Department of Labora-(HMWM) [2,3]. In this paper, we discuss an illustrative tory Medicine, Yale University School of Medicine, New Haven, CT case and review the recent literature on AvWD, which 06520-8035. details the physiologic basic for this disease and the ratioReceived for publication 9 August 1996; Accepted 11 September 1996. nale for treatment options.ᮊ 1997 Wiley-Liss, Inc.

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“…The mechanism of action is thought to be an anti-idiotype effect, blockage of reticulo-endothelial Fc-receptors, or elimination of circulating immune complexes by circulating immunoglobulin [20]. IVIG has been used successfully in patients with aVWS and IgG-type MGUS or lymphoproliferative disorders [20,[133][134][135][136][137] and, alone or in association with factor concentrates, for the treatment of active bleeding or for prophylaxis during surgical interventions or delivery in aVWS [23,[138][139][140][141][142][143]. Although data from the International Registry suggest a success rate of 33%, this tends to be higher when aVWS is associated with lymphoproliferative disorders, immune diseases, or neoplasia [15].…”

Section: Treatmentmentioning

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: Correction of hemostatic defect by high‐dose intravenous immunoglobulins

Cited by 38 publications

References 5 publications

Idiopathic immune-mediated acquired von Willebrand's disease in a patient with angiodysplasia: Demonstration of an unusual inhibitor causing a functional defect and rapid clearance of von Willebrand factor

Idiopathic immune-mediated acquired von Willebrand's disease in a patient with angiodysplasia: Demonstration of an unusual inhibitor causing a functional defect and rapid clearance of von Willebrand factor

Acquired von Willebrand's disease: A concise review

Acquired von Willebrand syndrome: An update

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