Acquired Von Willebrand’s Syndrome in Systemic Lupus Erythematosus

Abstract

Acquired von Willebrand syndrome (AVWS) is an uncommon, underdiagnosed, and heterogeneous disease which is increasingly recognized as a cause of bleeding diatheses. Systemic lupus erythematosus (SLE) is an infrequent cause of AVWS. Herein, we report a case of AVWS diagnosed during the initial presentation of SLE in a previously healthy young man with no family history of bleeding diathesis who presented with worsening epistaxis, gastrointestinal bleeding, and anasarca. He was found to have severe anemia and prolonged activated partial thromboplastin time (aPTT) with severely decreased levels of von Willebrand factor (VWF) measurements in addition to markedly decreased factor VIII levels. Further evaluation revealed nephrotic syndrome and interstitial lung disease due to SLE. He initially received combination therapy with intravenous immunoglobulin (IVIG) and von Willebrand factor/factor VIII concentrates without significant improvement. Treatment with steroids, cyclophosphamide, and rituximab was followed by clinical improvement evidenced by cessation of bleeding. The short follow-up did not allow us to definitely prove the therapeutic effect of immunosuppressive treatment on AVWS in SLE patients. This case adds to the literature supporting the relationship between AVWS and SLE and highlights the importance of combination therapy in the treatment of severe AVWS as well as the role of IVIG, cyclophosphamide, and rituximab in AVWS associated with SLE.

Severe acquired von Willebrand syndrome secondary to systemic lupus erythematosus

“…2 The first case of AVWS secondary to systemic lupus erythematosus (SLE) was reported by Simone et al 3 in 1968. Although this association is infrequently described in literature, 1,3,4 even less frequent is its presentation with clinical and laboratory features resembling a severe VWD phenotype. 5 Most cases have mild to moderate bleeding tendency, with laboratory characteristics similar to inherited VWD type 1.…”

“…Until the 1989 discovery of HCV, 3 almost all haemophilia patients treated with clotting factor products were infected with HCV. 4 The clinical presentation of HCV is characterized by chronic liver disease that can progress to fibrosis with development of (de)compensated cirrhosis and hepatocellular carcinoma. Haemophilia A murine models that mimic patients with severe haemophilia A are currently available.…”

Case report: A case of acquired von Willebrand syndrome as onset clinical presentation of systemic lupus erythematosus manifested as epistaxis and pulmonary hemorrhage

“…For AVWS and SLE, steroids and cyclophosphamide are effective in controlling hemorrhage (25-28). Recent studies have reported that rituximab is used to treat AVWS in adolescent SLE (29)(30)(31), and bleeding in both patients has been effectively controlled. In this case, the activity of VWF and FVIII returned to normal levels after methylprednisolone pulse therapy.…”

Splenic Marginal Zone Lymphoma with Acquired von Willebrand Syndrome Diagnosed via Splenic Bleeding

“…In the present case, although SMZL was quite advanced, the patient refused to receive conventional chemotherapy and instead chose rituximab monotherapy. In the literature, rituximab has not been effective in AVWS associated with MGUS (15-17) or with lymphoplasmacytic lymphoma (18), although it has been effective in AVWS associated with B cell lymphoma (MALT lymphoma) (10,11), monoclonal B cell lymphocytosis (19), essential mixed cryoglobulinemia (20), and SLE (21,22). Although the lymphadenopathy in our case showed mild shrinkage, the AVWS was not resolved.…”