Acquired hemophilia A and von Willebrand syndrome in a patient with late-onset systemic lupus erythematosus
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Acquired hemophilia A and von Willebrand syndrome in a patient with late-onset systemic lupus erythematosus. / Dicke, Christina; Holstein, Katharina; Schneppenheim, Sonja; Dittmer, Rita; Schneppenheim, Reinhard; Bokemeyer, Carsten; Iking-Konert, Christof; Budde, Ulrich; Langer, Florian.
In: EXP HEMATOL ONCOL, Vol. 3, 20.08.2014, p. 21.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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T1 - Acquired hemophilia A and von Willebrand syndrome in a patient with late-onset systemic lupus erythematosus
AU - Dicke, Christina
AU - Holstein, Katharina
AU - Schneppenheim, Sonja
AU - Dittmer, Rita
AU - Schneppenheim, Reinhard
AU - Bokemeyer, Carsten
AU - Iking-Konert, Christof
AU - Budde, Ulrich
AU - Langer, Florian
PY - 2014/8/20
Y1 - 2014/8/20
N2 - 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.
AB - 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.
U2 - 10.1186/2162-3619-3-21
DO - 10.1186/2162-3619-3-21
M3 - SCORING: Journal article
C2 - 25170428
VL - 3
SP - 21
JO - EXP HEMATOL ONCOL
JF - EXP HEMATOL ONCOL
SN - 2162-3619
ER -