title: Splenic Marginal Zone Lymphoma with Acquired von Willebrand Syndrome Diagnosed via Splenic Bleeding.
abstract: 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.
authors.affiliation: Department of Hematology, Japan Community Healthcare Organization (JCHO) Tokyo Yamate Medical Center, Japan.
authors.forename: Yukiko
authors.initials: Y
authors.lastname: Komeno
authors.forename: Naoki
authors.initials: N
authors.lastname: Shibuya
authors.forename: Hideki
authors.initials: H
authors.lastname: Uryu
authors.forename: Haruki
authors.initials: H
authors.lastname: Yamada
authors.forename: Takeo
authors.initials: T
authors.lastname: Toda
authors.forename: Masayuki
authors.initials: M
authors.lastname: Shibasaki
authors.forename: Shinji
authors.initials: S
authors.lastname: Kunishima
authors.forename: Kuniko
authors.initials: K
authors.lastname: Iihara
authors.forename: Tomiko
authors.initials: T
authors.lastname: Ryu
mesh_terms: D000369:Aged, 80 and over; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001777:Blood Coagulation; D005260:Female; D006470:Hemorrhage / Q000209:etiology*; D006801:Humans; D018442:Lymphoma, B-Cell, Marginal Zone / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D010314:Partial Thromboplastin Time; D000069283:Rituximab / Q000627:therapeutic use; D013156:Splenectomy; D013158:Splenic Diseases / Q000209:etiology; D013160:Splenic Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D014842:von Willebrand Diseases / Q000175:diagnosis / Q000209:etiology*
chemical_list: D000970:Antineoplastic Agents; D000069283:Rituximab