Acquired von Willebrand’s Syndrome in Systemic Lupus Erythematosus

Simone, Joseph V.; Cornet, Jo Ann; Abildgaard, Charles F.

doi:10.1182/blood.v31.6.806.806

Cited by 193 publications

(74 citation statements)

References 7 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Acquired von Willebrand disease was first described in a case of systematic lupus erythematosus (SLE) in 1968 (56). It is mainly associated with the development of autoantibodies against VWF.…”

Section: Immune-mediated Etiologymentioning

confidence: 99%

Pathophysiology of acquired von Willebrand disease: a concise review

Shetty

Kasatkar

Ghosh

2011

European Journal of Haematology

View full text Add to dashboard Cite

Acquired von Willebrand disease (AVWD) is a rare, underdiagnosed hemorrhagic disorder, which is similar to congenital VWD with regard to the clinical and laboratory parameters; however, it is found in individuals with no positive family history and has no genetic basis. The etiology is varied, the commonest being hematoproliferative disorders and cardiovascular disorders. Other disorders associated with AVWD are autoimmune disorders such as systematic lupus erythematosus, hypothyroidism, and neoplasia, or it may also be drug induced. In quite a few cases, the etiology is unknown. The pathogenic mechanisms are different in different underlying disorders or they may be overlapping among these disorders. Some of the proposed mechanisms include the development of autoantibodies, selective absorption of high molecular weight von Willebrand factor (VWF) multimers, non‐selective absorption of VWF, mechanical destruction of VWF under high shear stress, and increased proteolysis. This report presents a concise review of the pathophysiological mechanisms of AVWD in these various underlying conditions.

show abstract

Section: Immune-mediated Etiologymentioning

confidence: 99%

Pathophysiology of acquired von Willebrand disease: a concise review

Shetty

Kasatkar

Ghosh

2011

European Journal of Haematology

View full text Add to dashboard Cite

show abstract

“…It is characterized by mucocutaneous and/or gastrointestinal bleeding and a defect in vWF activity, estimated by ristocetin cofactor activity (vWF:RCo) and/or collagen binding activity (vWF:CBA). Up to now, more than 300 cases of AvWS have been reported in the literature since the first description of a case associated with systemic lupus erythematosus in 1968 [5]. However, this number is likely an underestimate because isolated case reports of this syndrome are less likely to be published.…”

Section: Introductionmentioning

confidence: 99%

Acquired von Willebrand syndrome: Features and management

Mohri

2006

Am. J. Hematol.

View full text Add to dashboard Cite

“…The four patients with abnormal bleeding described in this study have in common a long bleeding time, a low factor-VIII level and, in the three patients tested in this respect, more or less reduced platelet adhesiveness. Two patients showed a moderate, 'von Willebrand' type of response to the infusion of plasma; but we feel that thc disorder in all four patients is clearly distinguished from classical von Willebrand's disease by the evidence of onset late in life, and we follow Simone et al(1968) in referring to 'von Willebrand's syndrome'.…”

Section: Discussionmentioning

confidence: 70%

Four Cases of Acquired von Willebrand's Syndrome

et al. 1971

View full text Add to dashboard Cite

Four cases are reported in which a bleeding disorder associated with a long bleeding time and a low factor-VIII Icvel, like von Willebrand's disease, appears to have developed in adult life. In each there were abnormalities of plasma proteins, similar to but in some instances more extreme than patterns found in hacmophilia and 'classical' von Willebrand's disease. One patient's bleeding tendency developed in association with a generalized illness, probably systemic lupus erythematosus, and improved rapidly on steroid treatment. The other three patients, with no symptoms of underlying disease, were not treated with steroids. Two patients, infused with plasma, showed rather more than the predicted rise in factor VIII. 'Classical' von Willebrand's discase is a familial, life-long bleeding disorder with a long blecding time and a low level of factor VIII. However, Simone et al(1968) described a similar but acquircd disorder arising in a child with systemic lupus erythematosus (SLE) in whom both conditions improved on steroid therapy; and Veltkamp et al(1g70) observed a like episode lasting 3 wceks in a man who drank a pesticide. This paper describes four unrelated cases in which a bleeding disorder with some laboratory fcatures of von Willebrand's disease developed spontaneously in adult life in patients who are known previously to have responded normally to major haelnostatic challenges. MATERIALS AND METHODS PaticntsThe four patients studied were: H.S., female, 60 yr, Mayo Clinic; E.Ca., male, 76 yr and H.G., male, 5 5 yr, St Thomas's Hospital, London; J.Co., male, 59 yr, Royal South Hams Hospital, Southampton. They were unrclated and none gave a family history of abnormal bleeding. Each proffered clear evidence of normal liaemostasis for at least the first four decades of life. The onset of the bleeding disorder became apparent from a change in the haeniostatic response to trauma, and E.Ca., H.G. and J.Co. also developed spontaneous blceding of the 'capillary' type, characterized by ecchymoses and bleeding from mucous membranes (Table I), for which no local cause was identified in any case.

show abstract

Acquired von Willebrand’s Syndrome in Systemic Lupus Erythematosus

Cited by 193 publications

References 7 publications

Pathophysiology of acquired von Willebrand disease: a concise review

Pathophysiology of acquired von Willebrand disease: a concise review

Acquired von Willebrand syndrome: Features and management

Four Cases of Acquired von Willebrand's Syndrome

Contact Info

Product

Resources

About