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- Mai Owari, Kayo Harada-Shirado, Ryuichi Togawa, Masahiko Fukatsu, Yuki Sato, Koichiro Fukuchi, Mamiko Endo, Hiroshi Takahashi, Satoshi Kimura, Tsukasa Osaki, Masayoshi Souri, Akitada Ichinose, Yoko Shibata, Yuko Hashimoto, and Takayuki Ikezoe.
- Department of Hematology, Fukushima Medical University, Japan.
- Intern. Med. 2023 Feb 15; 62 (4): 605611605-611.
AbstractAcquired 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 life-threatening 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.
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