A case of essential mixed cryoglobulinemia and associated acquired von-Willebrand disease treated with rituximab

Abstract

Current treatment options of essential mixed cryoglobulinemia (EMC); include immunosuppressive approaches, such as corticosteroids, cyclophosphamide, plasma exchange, other cytotoxic drugs in moderate to severe manifestations. Some controlled studies have been carried out to assess the efficacy of anti-CD20 monoclonal antibody, rituximab in patients with hepatitis C (HCV) related cryoglobulinemia (CG) and in patients with autoimmune disorders. Recent trials and some case reports demonstrate a beneficial role for rituximab in HCV related mixed CG. Although, the published evidence for treatment of EMC with rituximab is restricted to case reports, which have shown positive results. Several diseases include lymphoproliferative and myeloproliferative disorders, solid tumors, immunological disorders, cardiovascular disorders and some drugs associated with acquired von Willebrand disease (avWD). CG, which is a kind of immune complex disease, may be related with development of autoantibodies to various autoantigens. In this present case report, we showed the efficacy of rituximab in a 21-year-old female patient, suffered from neuropathy and arthralgia related with EMC, and developed avWD, presented with mucosal bleeding associated with CG. von Willebrand factor activity of our patient also increased with controlling the underlying disease, EMC by rituximab. This case demonstrate that rituximab may be an effective treatment option in EMC and avWD mainly related to CG.

Maintaining hemostasis in acquired von Willebrand syndrome: a review of intravenous immunoglobulin and the importance of rituximab dose scheduling

“…Serendipitous remissions of AVWS after rituximab treatment for two cases of low‐grade lymphoma and a single case of Type III cryoglobulinemia have been reported . Furthermore, given the efficacy of rituximab for CLL‐associated autoimmune hemolytic anemia or autoimmune thrombocytopenia, the success in our case is not surprising .…”

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.…”

Current Diagnostic and Therapeutic Approaches to Patients with Acquired von Willebrand Syndrome: A 2013 Update

“…Other miscellaneous conditions associated with AVWS are listed in ►Table 4, and several references provide data on such patients. [60][61][62][63][64][65][66][67][68][69][70][71][72] In the data from the ISTH-SSC registry, 57% of the patients were classified as bleeders and the risk of new bleeding episodes was 27%/year; 10 Notably, in 27% of the patients, surgical procedures were required within 1 year after diagnosis. 17 Because of the high risk of new bleeding episodes and the necessity to make plans for controlling bleeding with surgery for many patients in this group, it is important to establish their AVWS diagnosis.…”