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suck abstract from ncbi


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pmid28250305      Intern+Med 2017 ; 56 (5): 557-62
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  • Splenic Marginal Zone Lymphoma with Acquired von Willebrand Syndrome Diagnosed via Splenic Bleeding #MMPMID28250305
  • Komeno Y; Shibuya N; Uryu H; Yamada H; Toda T; Shibasaki M; Kunishima S; Iihara K; Ryu T
  • Intern Med 2017[Mar]; 56 (5): 557-62 PMID28250305show ga
  • An 85-year-old woman underwent emergent splenectomy due to left abdominal pain and active bleeding in a massively enlarged spleen. The histological diagnosis was splenic marginal zone lymphoma (SMZL). A prolonged activated partial thromboplastin time (APTT) was noted, and additional tests led to the diagnosis of type 2A-like acquired von Willebrand syndrome (AVWS). An APTT cross mixing test ruled out the presence of inhibitors. She received eight courses of rituximab monotherapy. The coagulation data showed no improvement, possibly because the lymphoma showed a poor response to the treatment. AVWS rarely causes bleeding in solid organs. This is the first case of SMZL with AVWS diagnosed via splenic bleeding.
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