Current Diagnostic and Therapeutic Approaches to Patients with Acquired von Willebrand Syndrome: A 2013 Update

Federici, Augusto B.; Budde, Ulrich; Castaman, Giancarlo; Rand, Jacob H.; Tiede, Andreas

doi:10.1055/s-0033-1334867

Cited by 117 publications

(69 citation statements)

References 62 publications

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“…DDAVP and VWF concentrates agents have been used to control bleeding, although these treatments can be subject to rapid clearance. Immunosuppression has also been used to treat the underlying MGUS syndrome 88. The MGUS AVWS subtype appears to respond to intravenous immunoglobulins (IVIG) that binds the autoantibody.…”

Section: Acquired Avws and Agingmentioning

confidence: 99%

Von Willebrand Disease in the elderly: clinical perspectives

Chapin

2018

CIA

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Von Willebrand disease (VWD) is an inherited bleeding disorder that affects up to 1% of the population. In most cases, VWD results from a mutation in the von Willebrand Factor (VWF) gene, which alters the amount and function of VWF, a key glycoprotein in both primary and secondary hemostasis. A comprehensive analysis of patients with VWD should include VWF activity, antigen levels, platelet function, and a careful bleeding history. Treatment options include antifibrinolytics, desmopressin, and VWF replacement therapy. VWF levels fluctuate due to age, stress, environmental exposures, and pharmacologic treatment. Treatment guidelines exist to treat and prevent bleeding for patients undergoing surgery and medical procedures, but often these must be reevaluated in the setting of age-related comorbidities including cardiovascular events, venous thrombosis, and malignancy. In addition, many age-related complications are associated with a secondary acquired von Willebrand syndrome (AVWS), including malignancies, hypothyroidism, cardiovascular diseases, and cardiac replacement devices. The current literature is limited by a lack of older patients in clinical trials. Larger studies are needed to determine if age-related comorbidities affect VWD patients at different frequencies than the general elderly population. There is also a significant need for registry-based studies to evaluate many age-related comorbidities in VWD patients.

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Section: Acquired Avws and Agingmentioning

confidence: 99%

Von Willebrand Disease in the elderly: clinical perspectives

Chapin

2018

CIA

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“…Furthermore, the management of AHA remains difficult and the costs of treatment are often immense. Although AHA is thus clinically and economically an important disorder, it is often unrecognized or misdiagnosed as other acquired hemorrhagic disorders, such as disseminated intravascular coagulation (DIC) and acquired inhibitors against von Willebrand factor (acquired von Willebrand syndrome [9]) and factor XIII (acquired factor XIII deficiency [10]).…”

Section: Introductionmentioning

confidence: 99%

Acquired Hemophilia A: A Frequently Overlooked Autoimmune Hemorrhagic Disorder

Sakurai

Takeda

2014

Journal of Immunology Research

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Acquired hemophilia A (AHA) is a rare hemorrhagic disease in which autoantibodies against coagulation factor VIII- (FVIII-) neutralizing antibodies (inhibitors) impair the intrinsic coagulation system. As the inhibitors developed in AHA are autoantibodies, the disease may have an autoimmune cause and is often associated with autoimmune disease. Although acute hemorrhage associated with AHA may be fatal and is costly to treat, AHA is often unrecognized or misdiagnosed. AHA should thus be considered in the differential diagnosis particularly in postpartum women and the elderly with bleeding tendency or prolonged activated partial thromboplastin time. Cross-mixing tests and measurement of FVIII-binding antibodies are useful to confirm AHA diagnosis. For treatment of acute hemorrhage, hemostatic therapy with bypassing agents should be provided. Unlike in congenital hemophilia A with inhibitors, in which immune tolerance induction therapy using repetitive infusions of high-dose FVIII concentrates is effective for inhibitor eradication, immune tolerance induction therapy has shown poor efficacy in treating AHA. Immunosuppressive treatment should thus be initiated to eradicate inhibitors as soon as the diagnosis of AHA is confirmed.

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“…In contrast, virtually all cases of AVWS are secondary to another disease, including hematological, lymphoproliferative, cardiovascular, and autoimmune disorders as well as hypothyroidism [2-4]. In this regard, it is noteworthy that the first description of AVWS has been in a boy suffering from systemic lupus erythematosus (SLE) [5] with subsequent studies clearly implicating the production of VWF autoantibodies in the pathogenesis of SLE-associated AVWS [6-11].…”

Section: Introductionmentioning

confidence: 99%

Acquired hemophilia A and von Willebrand syndrome in a patient with late-onset systemic lupus erythematosus

et al. 2014

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Acquired hemophilia A (AHA) and acquired von Willebrand Syndrome (AVWS) are both rare bleeding disorders that can be associated with lymphoproliferative or autoimmune diseases. AHA is uniformly caused by inhibitory autoantibodies against coagulation factor VIII (FVIII), while the pathophysiology of AVWS comprises several distinct mechanisms, including reduced synthesis, accelerated clearance, or increased proteolysis. In this regard, autoantibodies to von Willebrand factor (VWF) have been described in patients with systemic lupus erythematosus (SLE) or monoclonal gammopathy. Here, we report the case of a 71-year-old patient with a recent onset of spontaneous mucocutaneous and soft-tissue bleeding due to severely decreased FVIII and VWF. While there was no evidence for monoclonal gammopathy, specific IgG antibodies against both FVIII and VWF were detected. Furthermore, VWF multimer analysis revealed the presence of ultralarge plasma multimers and absence of the typical multimeric triplet structure, a finding consistent with decreased proteolytic processing of massively released, but rapidly cleared VWF. Both FVIII and VWF readily responded to immunosuppressive therapy with prednisolone. Interestingly, clinical and laboratory findings established the diagnosis of “late-onset SLE” in our patient. Thus, about 45 years after the first description of AVWS in a 12-year-old boy with SLE, we present another unusual case of concomitant autoimmune-mediated AHA and AVWS in an elderly SLE patient, which, to the best of our knowledge, has not been reported so far.

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Current Diagnostic and Therapeutic Approaches to Patients with Acquired von Willebrand Syndrome: A 2013 Update

Cited by 117 publications

References 62 publications

Von Willebrand Disease in the elderly: clinical perspectives

Von Willebrand Disease in the elderly: clinical perspectives

Acquired Hemophilia A: A Frequently Overlooked Autoimmune Hemorrhagic Disorder

Acquired hemophilia A and von Willebrand syndrome in a patient with late-onset systemic lupus erythematosus

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