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Experimental Hematology and Oncology

, 3:21

First Online: 20 August 2014Received: 02 July 2014Accepted: 04 August 2014DOI: 10.1186-2162-3619-3-21

Cite this article as: Dicke, C., Holstein, K., Schneppenheim, S. et al. Exp Hematol Oncol 2014 3: 21. doi:10.1186-2162-3619-3-21


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.

KeywordsAcquired von Willebrand syndrome Acquired hemophilia A Systemic lupus erythematosus Ultralarge von Willebrand factor plasma multimers AbbreviationsAHAAcquired hemophilia A

AVWSAcquired von Willebrand syndrome

VWFVon Willebrand factor


SLESystemic lupus erythematosus

APTTActivated partial thromboplastin time

FVIIIC: Factor VIII clotting activity

ELISAEnzyme-linked immunosorbent assay

BUBethesda units

ODOptical densities

PFAPlatelet function analyzer

VWFAg: Von Willebrand factor antigen

VWFAc: Von Willebrand factor activity

rhVWFRecombinant human von Willebrand factor

CHOChinese hamster ovary

ANAAntinuclear antibodies

MGUSMonoclonal gammopathy of unknown significance

TTPThrombotic-thrombocytopenic purpura.

Electronic supplementary materialThe online version of this article doi:10.1186-2162-3619-3-21 contains supplementary material, which is available to authorized users.

Christina Dicke, Katharina Holstein contributed equally to this work.

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Autor: Christina Dicke - Katharina Holstein - Sonja Schneppenheim - Rita Dittmer - Reinhard Schneppenheim - Carsten Bokemeyer - Chr


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