Treatment of Acquired von Willebrand Syndrome and Prevention of Bleeding Postautologous Stem Cell Transplant during Severe Pancytopenia with IVIG

Abstract

The use of high dose chemotherapy followed by autologous hematopoietic stem cell transplantation for remission consolidation after initial induction represents standard of care for patients with multiple myeloma. Patients with myeloma and Acquired von Willebrand Syndrome (AVWS) undergoing autologous stem cell transplant (ASCT) are at significant risk of bleeding due to the profound thrombocytopenia, low Factor VIII levels, fever, and toxicities associated with the preparative regimen. We report a patient with AVWS associated with multiple myeloma who underwent autologous stem cell transplants as consolidation after initial induction and again at relapse. He was successfully treated with high dose intravenous immunoglobulin (IVIG) prior to each transplant with rapid resolution of AVWS.

Acquired von Willebrand syndrome and lymphoproliferative disorders: A case report

“…To our knowledge, ours is an unprecedented use of daratumumab in a patient with MM‐associated AWS . Our results have potentially important implications in guiding the treatment of patients with asymptomatic plasma cell disorder‐associated AWS.…”

Acquired von Willebrand syndrome and lymphoid neoplasms: A review of malignancy management, and propositions of practical recommendations

“…Eight patients benefited from autologous HSCT in PRD, including two patients who underwent two autologous HSCTs 25–27,30,31,33,34 . No severe bleeding was reported.…”

Successful treatment of acquired von Willebrand disease with lenalidomide leading to dramatic resolution of intractable gastrointestinal bleeding: A case report

“…LETTERS TO THE EDITOR first case, a 66-year-old patient had multiple myeloma with AVWD (the AVWD responded to IVIG) and lenalidomide was used for the treatment of the MM rather than the AVWD, with an increase in the VWF after IVIG. 1 Two other patients have been reported; a 75-year-old man with IgG MGUS who had significance response in VWF levels and reduction in bleeding with lenalidomide and a 63-year-old man with IgA multiple myeloma where the lenalidomide was used to treat the myeloma with a reduction in the paraprotein burden and subsequent sustained corresponding response in VWF levels. 2 In this case, the lenalidomide has not allowed VWF prophylaxis to be stopped, but has significantly reduced bleeding episodes.…”

“…to most estimates, there are approximately three to five potential female carriers for each male with haemophilia 1. While most women within each haemophilia family are assumed to have been screened for carrier status and its implications, this is presently still not the case.…”