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

Yukiko Komeno; Naoki Shibuya; Hideki Uryu; Haruki Yamada; Takeo Toda; Masayuki Shibasaki; Shinji Kunishima; Kuniko Iihara; Tomiko Ryu

doi:10.2169/internalmedicine.56.7258

CASE REPORTS

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

Yukiko Komeno, Naoki Shibuya, Hideki Uryu, Haruki Yamada, Takeo Toda, Masayuki Shibasaki, Shinji Kunishima, Kuniko Iihara, Tomiko Ryu

Author information

Yukiko Komeno
Department of Hematology, Japan Community Healthcare Organization (JCHO) Tokyo Yamate Medical Center, Japan
Naoki Shibuya
Department of Hematology, Japan Community Healthcare Organization (JCHO) Tokyo Yamate Medical Center, Japan
Hideki Uryu
Department of Hematology, Japan Community Healthcare Organization (JCHO) Tokyo Yamate Medical Center, Japan
Haruki Yamada
Department of Gastroenterology, JCHO Tokyo Yamate Medical Center, Japan
Takeo Toda
Department of Surgery, JCHO Tokyo Yamate Medical Center, Japan
Masayuki Shibasaki
Department of Surgery, JCHO Tokyo Yamate Medical Center, Japan
Shinji Kunishima
Clinical Research Center, Nagoya Medical Center, National Hospital Center, Japan
Kuniko Iihara
Department of Pathology, JCHO Tokyo Yamate Medical Center, Japan
Tomiko Ryu
Department of Hematology, Japan Community Healthcare Organization (JCHO) Tokyo Yamate Medical Center, Japan

Keywords: splenic marginal zone lymphoma (SMZL), splenic bleeding, acquired von Willebrand syndrome (AVWS), rituximab

JOURNAL OPEN ACCESS

2017 Volume 56 Issue 5 Pages 557-562

DOI https://doi.org/10.2169/internalmedicine.56.7258

Details

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.

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