Splenic Marginal Zone Lymphoma with Acquired von Willebrand Syndrome Diagnosed via Splenic Bleeding

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...

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Published inInternal Medicine Vol. 56; no. 5; pp. 557 - 562
Main Authors Komeno, Yukiko, Shibuya, Naoki, Uryu, Hideki, Yamada, Haruki, Toda, Takeo, Shibasaki, Masayuki, Kunishima, Shinji, Iihara, Kuniko, Ryu, Tomiko
Format Journal Article
LanguageEnglish
Published Japan The Japanese Society of Internal Medicine 01.01.2017
Japan Science and Technology Agency
Subjects
acquired von Willebrand syndrome (AVWS)
Aged, 80 and over
Antineoplastic Agents - therapeutic use
Bleeding
Blood Coagulation
Case Report
Diagnosis
Female
Health risk assessment
Hemorrhage - etiology
Humans
Internal medicine
Lymphoma
Lymphoma, B-Cell, Marginal Zone - complications
Lymphoma, B-Cell, Marginal Zone - diagnosis
Lymphoma, B-Cell, Marginal Zone - drug therapy
Mixing tests
Monoclonal antibodies
Organs
Pain
Partial Thromboplastin Time
Rituximab
Rituximab - therapeutic use
Spleen
Splenectomy
splenic bleeding
Splenic Diseases - etiology
splenic marginal zone lymphoma (SMZL)
Splenic Neoplasms - complications
Splenic Neoplasms - diagnosis
Splenic Neoplasms - drug therapy
Targeted cancer therapy
Thromboplastin
von Willebrand Diseases - diagnosis
von Willebrand Diseases - etiology
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Summary: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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Correspondence to Dr.　Yukiko Komeno, ykomeno-tky@umin.ac.jp
ISSN:0918-2918
1349-7235
DOI:10.2169/internalmedicine.56.7258