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Aggressive variant of splenic marginal zone lymphoma characterized using a cancer panel test and treated with rituximab-containing chemotherapy A case report

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Title: Aggressive variant of splenic marginal zone lymphoma characterized using a cancer panel test and treated with rituximab-containing chemotherapy A case report
Authors: Ishiguro, Kazuya Browse this author
Sasaki, Yasushi Browse this author
Takagi, Yoshitake Browse this author
Niinuma, Takeshi Browse this author
Suzuki, Hiromu Browse this author
Tokino, Takashi Browse this author
Hayashi, Toshiaki Browse this author
Takahashi, Tohru Browse this author
Igarashi, Tetsuyuki Browse this author
Matsuno, Yoshihiro Browse this author →KAKEN DB
Keywords: aggressive variant of splenic marginal zone lymphoma
cancer panel test
next generation sequencing
Issue Date: 28-Aug-2020
Publisher: Lippincott Williams & Wilkins (LWW)
Journal Title: Medicine
Volume: 99
Issue: 35
Publisher DOI: 10.1097/MD.0000000000021938
Abstract: Rationale: Aggressive variant of splenic marginal zone lymphoma (AV-SMZL) is a very rare disease that is often associated withTP53mutations and has a poor prognosis. On the other hand, recent advances in genome sequencing techniques enable us to understand the molecular characteristics of rare cancers such as AV-SMZL. Here we present a case of AV-SMZL analyzed using a genetic test. Patient Concerns: A 66-year-old woman was admitted with splenomegaly and lymphocytosis. Computed tomography revealed marked splenomegaly without lymphadenopathy in any other areas. The serum soluble interleukin-2 receptor (sIL-2R) level was significantly elevated. Peripheral and bone marrow blood tests showed an increase in abnormal lymphocytes. Diagnosis: A splenectomy revealed an SMZL pattern with increased numbers of large cells and mitotic cells and a high Ki-67 positivity rate, which led to a diagnosis of AV-SMZL. AlthoughTP53mutation was not detected, mutations inNOTCH2,NCOA4,PTEN,EPHA3,andKMT2Dwere identified. Among these, the mutations inNCOA4,PTEN,andEPHA3were novel pathogenic mutations in SMZL, which suggests they may be related to the aggressiveness and persistence of the disease. Interventions: The patient was administered a rituximab-containing regimen and rituximab-maintenance therapy. Outcomes: The patient continues to exhibit a complete response. Lessons: This is a case of AV-SMZL in which a cancer panel test successfully detected genetic alterations that are potentially associated with its pathogenesis. These findings suggest that genetic analysis is useful for making diagnoses as well as for determining treatment strategies in AV-SMZL.
Type: article
Appears in Collections:北海道大学病院 (Hokkaido University Hospital) > 雑誌発表論文等 (Peer-reviewed Journal Articles, etc)

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