A Case of CD5-/Cyclin D1+/SOX11- Mantle Cell Lymphoma with an Aberrant Immunophenotype and Indolent Clinical Course

Main Authors: Zhang, Lei, Zhang, Nan
Format: Article eJournal
Terbitan: , 2017
Subjects:
Online Access: https://zenodo.org/record/1157654
ctrlnum 1157654
fullrecord <?xml version="1.0"?> <dc schemaLocation="http://www.openarchives.org/OAI/2.0/oai_dc/ http://www.openarchives.org/OAI/2.0/oai_dc.xsd"><creator>Zhang, Lei</creator><creator>Zhang, Nan</creator><date>2017-05-29</date><description>Mantle cell lymphoma (MCL) is a clinically aggressive B-cell lymphoma associated with 11q13 translocation, which leads to cyclin D1 overexpression in almost all cases. Although CD5 expression is characteristic in MCL, rare CD5 negative cases with variable expression of CD10 and CD23 have been reported. Over the recent years, a subgroup of MCL with a relatively indolent clinical course started to be recognized. Herein, we report a case of CD5-/Cyclin D1+/SOX11- MCL in a 75-year-old female with diffuse and persistent lymphoma involving multiple lymph nodes, spleen, bone marrow, and lacrimal ducts over the course of nine years. Despite multiple chemotherapy regimens, the MCL had slowly progressed while her baseline health condition remained stable. The neoplastic lymphocytes from different time points during her clinical course showed similar histological features, genetic abnormality, and immunophenotypes. In particular, the lymphoma cells were CD5-, with overexpression of cyclin D1, aberrant expression of CD10 and BCL-6, absence of SOX11 expression, and presence of t(11; 14) (q13; q32) translocation. The indolent clinical course and unusual immunophenotype suggest this particular type of MCL may be considered a unique subentity under MCL. </description><description>transfer-of-copyright</description><identifier>https://zenodo.org/record/1157654</identifier><identifier>10.7156/najms.2017.1001040</identifier><identifier>oai:zenodo.org:1157654</identifier><rights>info:eu-repo/semantics/openAccess</rights><source>North American Journal of Medicine and Science 10(1)</source><subject>B-cell lymphoma</subject><subject>mantle cell lymphoma</subject><subject>CD5 negative mantle cell lymphoma</subject><subject>indolent mantle cell lymphoma</subject><title>A Case of CD5-/Cyclin D1+/SOX11- Mantle Cell Lymphoma with an Aberrant Immunophenotype and Indolent Clinical Course</title><type>Journal:Article</type><type>Journal:Article</type><recordID>1157654</recordID></dc>
format Journal:Article
Journal
Journal:eJournal
author Zhang, Lei
Zhang, Nan
title A Case of CD5-/Cyclin D1+/SOX11- Mantle Cell Lymphoma with an Aberrant Immunophenotype and Indolent Clinical Course
publishDate 2017
topic B-cell lymphoma
mantle cell lymphoma
CD5 negative mantle cell lymphoma
indolent mantle cell lymphoma
url https://zenodo.org/record/1157654
contents Mantle cell lymphoma (MCL) is a clinically aggressive B-cell lymphoma associated with 11q13 translocation, which leads to cyclin D1 overexpression in almost all cases. Although CD5 expression is characteristic in MCL, rare CD5 negative cases with variable expression of CD10 and CD23 have been reported. Over the recent years, a subgroup of MCL with a relatively indolent clinical course started to be recognized. Herein, we report a case of CD5-/Cyclin D1+/SOX11- MCL in a 75-year-old female with diffuse and persistent lymphoma involving multiple lymph nodes, spleen, bone marrow, and lacrimal ducts over the course of nine years. Despite multiple chemotherapy regimens, the MCL had slowly progressed while her baseline health condition remained stable. The neoplastic lymphocytes from different time points during her clinical course showed similar histological features, genetic abnormality, and immunophenotypes. In particular, the lymphoma cells were CD5-, with overexpression of cyclin D1, aberrant expression of CD10 and BCL-6, absence of SOX11 expression, and presence of t(11; 14) (q13; q32) translocation. The indolent clinical course and unusual immunophenotype suggest this particular type of MCL may be considered a unique subentity under MCL.
transfer-of-copyright
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