Mantle cell lymphoma

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Mantle cell lymphoma (MCL) is a type of non-Hodgkin lymphoma (NHL) and accounts for ~5% of all NHL. It is a malignant neoplasm of virgin B cells that closely resemble normal mantle zone B cells surrounding germinal centres.

Epidemiology

They occur in older adults (mean age ~60 years), and there is a recognised male predilection (M: F of ~4:1) ².

MCL is usually widespread at diagnosis and frequently involves, apart from lymph nodes, the spleen, bone marrow, and gastrointestinal tract.

Pathology

MCL is characterised by an infiltrate of small to medium-sized cells with folded nuclei and scant cytoplasm. The diagnosis of MCL is confirmed by histological assessment and immunohistochemical evaluation, including cyclin D1.

Genetics

Many cases have the t(11;14)(q13;q32) translocation that causes overexpression of cyclin-D1 ².

Staging

Staging is similar to standard non-Hodgkin lymphoma staging ~~and is as~~:

stage I: involvement of one lymph node or group of adjacent lymph nodes or one extra lymphatic site
stage II: involvement of two or more nodal groups on the same side of the diaphragm or stage I or II with contiguous extra-nodal involvement
stage III: involvement of lymph nodes on either side of the diaphragm
stage IV: involvement of bone marrow or non-contiguous extra-nodal involvement

Treatment and prognosis

Survival is short, with a ~50% 5-year survival. Over one-third of the patients can die within a year despite the administration of aggressive combination chemotherapy.

-<p><strong>Mantle cell lymphoma (MCL)</strong> is a type of <a href="/articles/non-hodgkin-lymphoma">non-Hodgkin lymphoma</a> (NHL) and accounts for ~5% of all NHL. It is a malignant neoplasm of virgin B cells that closely resemble normal mantle zone B cells surrounding germinal centres.</p><h4>Epidemiology</h4><p>They occur in older adults (mean age ~60 years), and there is a recognised male predilection (M: F of ~4:1) <sup>2</sup>.</p><p>MCL is usually widespread at diagnosis and frequently involves, apart from lymph nodes, the spleen, bone marrow, and gastrointestinal tract.</p><h4>Pathology</h4><p>MCL is characterised by an infiltrate of small to medium-sized cells with folded nuclei and scant cytoplasm. The diagnosis of MCL is confirmed by histological assessment and immunohistochemical evaluation, including cyclin D1.</p><h5>Genetics</h5><p>Many cases have the t(11;14)(q13;q32) translocation that causes overexpression of cyclin-D1 <sup>2</sup>.</p><h4>Staging</h4><p>Staging is similar standard <a href="/articles/non-hodgkin-lymphoma-staging" title="non-Hodgkin lymphoma staging">non-Hodgkin lymphoma staging</a> and is as</p><ul>
~~-<li><p><strong>stage I</strong>: involvement of one lymph node or group of adjacent lymph nodes or one extra lymphatic site </p></li>~~
~~-<li><p><strong>stage II:</strong> involvement of two or more nodal groups on the same side of the diaphragm or stage I or II with contiguous extra-nodal involvement</p></li>~~
~~-<li><p><strong>stage III</strong>: involvement of lymph nodes on either side of the diaphragm</p></li>~~
~~-<li><p><strong>stage IV</strong>: involvement of bone marrow or non-contiguous extra-nodal involvement</p></li>~~
-</ul><p>See also: <a href="/articles/lugano-staging-classification-1" title="Lugano staging classification">Lugano staging classification</a></p><h4>Treatment and prognosis</h4><p>Survival is short, with a ~50% 5-year survival. Over one-third of the patients can die within a year despite the administration of aggressive combination chemotherapy.</p>
+<p><strong>Mantle cell lymphoma (MCL)</strong> is a type of <a href="/articles/non-hodgkin-lymphoma" title="Non-Hodgkin lymphoma (NHL)">non-Hodgkin lymphoma (NHL)</a> and accounts for ~5% of all NHL. It is a malignant neoplasm of virgin B cells that closely resemble normal mantle zone B cells surrounding germinal centres.</p><h4>Epidemiology</h4><p>They occur in older adults (mean age ~60 years), and there is a recognised male predilection (M: F of ~4:1) <sup>2</sup>.</p><p>MCL is usually widespread at diagnosis and frequently involves, apart from lymph nodes, the spleen, bone marrow, and gastrointestinal tract.</p><h4>Pathology</h4><p>MCL is characterised by an infiltrate of small to medium-sized cells with folded nuclei and scant cytoplasm. The diagnosis of MCL is confirmed by histological assessment and immunohistochemical evaluation, including cyclin D1.</p><h5>Genetics</h5><p>Many cases have the t(11;14)(q13;q32) translocation that causes overexpression of cyclin-D1 <sup>2</sup>.</p><h4>Staging</h4><p>Staging is similar to standard <a href="/articles/non-hodgkin-lymphoma-staging" title="non-Hodgkin lymphoma staging">non-Hodgkin lymphoma staging</a>:</p><ul>
+<li><p><strong>stage I</strong>: involvement of one lymph node or group of adjacent lymph nodes or one extra lymphatic site </p></li>
+<li><p><strong>stage II:</strong> involvement of two or more nodal groups on the same side of the diaphragm or stage I or II with contiguous extra-nodal involvement</p></li>
+<li><p><strong>stage III</strong>: involvement of lymph nodes on either side of the diaphragm</p></li>
+<li><p><strong>stage IV</strong>: involvement of bone marrow or non-contiguous extra-nodal involvement</p></li>
+</ul><p>See also: <a href="/articles/lugano-staging-classification-1" title="Lugano staging classification">Lugano staging classification</a></p><h4>Treatment and prognosis</h4><p>Survival is short, with a ~50% 5-year survival. Over one-third of the patients can die within a year despite the administration of aggressive combination chemotherapy.</p>

References changed:

1. Brepoels L, Stroobants S, De Wever W et al. Positron Emission Tomography in Mantle Cell Lymphoma. Leuk Lymphoma. 2008;49(9):1693-701. <a href="https://doi.org/10.1080/10428190802216707">doi:10.1080/10428190802216707</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/18798104">Pubmed</a>
2. Pérez-Galán P, Dreyling M, Wiestner A. Mantle Cell Lymphoma: Biology, Pathogenesis, and the Molecular Basis of Treatment in the Genomic Era. Blood. 2011;117(1):26-38. <a href="https://doi.org/10.1182/blood-2010-04-189977">doi:10.1182/blood-2010-04-189977</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/20940415">Pubmed</a>
3. Chung H, Kim Y, Kim J et al. Imaging Findings of Mantle Cell Lymphoma Involving Gastrointestinal Tract. Yonsei Med J. 2003;44(1):49-57. <a href="https://doi.org/10.3349/ymj.2003.44.1.49">doi:10.3349/ymj.2003.44.1.49</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/12619175">Pubmed</a>
1. Brepoels L, Stroobants S, De wever W et-al. Positron emission tomography in mantle cell lymphoma. Leuk. Lymphoma. 2008;49 (9): 1693-701. <a href="http://dx.doi.org/10.1080/10428190802216707">doi:10.1080/10428190802216707</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/18798104">Pubmed citation</a><div class="ref_v2"></div>
2. Pérez-galán P, Dreyling M, Wiestner A. Mantle cell lymphoma: biology, pathogenesis, and the molecular basis of treatment in the genomic era. 2010;<a href="http://dx.doi.org/10.1182/blood-2010-04-189977">doi:10.1182/blood-2010-04-189977</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/20940415">Pubmed citation</a><div class="ref_v2"></div>
3. Chung HH, Kim YH, Kim JH et-al. Imaging findings of mantle cell lymphoma involving gastrointestinal tract. Yonsei Med. J. 2003;44 (1): 49-57. <a href="http://www.eymj.org/DOIx.php?id=10.3349/ymj.2003.44.1.49">Yonsei Med. J. (link)</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/12619175">Pubmed citation</a><div class="ref_v2"></div>

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