title: Acquired hemophilia A and von Willebrand syndrome in a patient with late-onset systemic lupus erythematosus.
abstract: 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.
authors.affiliation: II. Medizinische Klinik und Poliklinik, Universitätsklinikum Eppendorf, Martinistr. 52, 20246 Hamburg, Germany.
authors.forename: Christina
authors.initials: C
authors.lastname: Dicke
authors.affiliation: II. Medizinische Klinik und Poliklinik, Universitätsklinikum Eppendorf, Martinistr. 52, 20246 Hamburg, Germany.
authors.forename: Katharina
authors.initials: K
authors.lastname: Holstein
authors.affiliation: Gerinnungslabor, MEDILYS Laborgesellschaft mbH, c/o Asklepios Klinik Altona, Paul-Ehrlich-Str. 1, 22763 Hamburg, Germany.
authors.forename: Sonja
authors.initials: S
authors.lastname: Schneppenheim
authors.affiliation: Gerinnungslabor, MEDILYS Laborgesellschaft mbH, c/o Asklepios Klinik Altona, Paul-Ehrlich-Str. 1, 22763 Hamburg, Germany.
authors.forename: Rita
authors.initials: R
authors.lastname: Dittmer
authors.affiliation: Klinik und Poliklinik für Pädiatrische Hämatologie und Onkologie, Universitätsklinikum Eppendorf, Martinistr. 52, 20246 Hamburg, Germany.
authors.forename: Reinhard
authors.initials: R
authors.lastname: Schneppenheim
authors.affiliation: II. Medizinische Klinik und Poliklinik, Universitätsklinikum Eppendorf, Martinistr. 52, 20246 Hamburg, Germany.
authors.forename: Carsten
authors.initials: C
authors.lastname: Bokemeyer
authors.affiliation: III. Medizinische Klinik und Poliklinik, Universitätsklinikum Eppendorf, Martinistr. 52, 20246 Hamburg, Germany.
authors.forename: Christof
authors.initials: C
authors.lastname: Iking-Konert
authors.affiliation: Gerinnungslabor, MEDILYS Laborgesellschaft mbH, c/o Asklepios Klinik Altona, Paul-Ehrlich-Str. 1, 22763 Hamburg, Germany.
authors.forename: Ulrich
authors.initials: U
authors.lastname: Budde
authors.affiliation: II. Medizinische Klinik und Poliklinik, Universitätsklinikum Eppendorf, Martinistr. 52, 20246 Hamburg, Germany.
authors.forename: Florian
authors.initials: F
authors.lastname: Langer
keywords: Acquired hemophilia A; Acquired von Willebrand syndrome; Systemic lupus erythematosus; Ultralarge von Willebrand factor plasma multimers