Acquired von Willebrand Syndrome in a Patient with Multiple Comorbidities, Including MALT Lymphoma with IgA Monoclonal Gammopathy and Hyperviscosity Syndrome

Abstract

Acquired von Willebrand syndrome (aVWS) develops with various underlying diseases. We herein report an individual with aVWS associated with mucosa-associated lymphoid tissue lymphoma in the lungs complicated by hyperviscosity syndrome, Sjögren's syndrome, and hypothyroidism. This patient developed lifethreatening hemorrhaging during a lung biopsy despite transfusion of concentrate of plasma-derived VWF/ factor VIII. The use of rituximab caused remission of the lymphoma and hyperviscosity syndrome in parallel with the resolution of aVWS. Thus, lymphoma and hyperviscosity might result in aVWS. Invasive procedures with a risk of bleeding should be avoided in individuals with aVWS.

Rare primary pulmonary mucosa-associated lymphoid tissue lymphoma misdiagnosed with tuberculosis: A case report

“…[7] The disease is recognized as a unique extranodal marginal zone lymphoma according to the World Health Organization (WHO) classification. [8] Pulmonary MALToma has recently been reported to have a median age of approximately 60 years at diagnosis [9] and an equal incidence rate in males and females. [10]…”

“…[7] The disease is recognized as a unique extranodal marginal zone lymphoma according to the World Health Organization (WHO) classification. [8] Pulmonary MALToma has recently been reported to have a median age of approximately 60 years at diagnosis [9] and an equal incidence rate in males and females. [10] The etiology that could play a role in the pathogenesis of pulmonary MALToma is yet to be completely elucidated, but it is currently widely accepted that immune cross-reactions caused by chronic inflammation (including smoking, autoimmune stimuli, and infectious agents such as bacteria and viruses) participate in this process.…”

Autoimmune Diseases Affecting Hemostasis: A Narrative Review

“…Additional common mechanisms of AVWS include absorption of VWF on malignant cells, reduced production (e.g., as in hypothyroidism), and increased blood platelet count such as in essential thrombocythemia. Nevertheless, a proportion of AVWS cases do have an autoimmune basis (e.g., arise in autoimmune disorders such as SLE, scleroderma, and APS), or else are associated with production of autoantibodies against VWF [ 52 , 53 ].…”

Sustained good response to rituximab in acquired von Willebrand syndrome

“…There is a reported 60% response rate to rituximab when considering treatment of AvWS associated with lymphoid disorders [2]. Cases of AvWS associated with monoclonal B lymphocytosis [3] and MALT lymphoma [4][5][6] are reported with a good response to rituximab, with some patients receiving monotherapy, which resulted in normalization of the coagulation profile. Gastrointestinal bleeding associated with angiodysplasia and AvWS has been treated successfully with rituximab after intravenous immunoglobulin adherence proved difficult [7].…”