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How is mantle cell lymphoma different from other lymphomas?
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JiaRuanMDPhD (Physician - Oncology - Hematology/Oncology (Verified) ) - 08 / 04 / 2012
Mantle cell lymphoma (MCL) is a rare but distinct subtype of B-cell non-Hodgkin’s lymphoma (NHL), representing 5-8% of NHL. Pathologically, it has a characteristic genetic translocation t(11;14)(q13;q32) which overproduces cyclin D1, a cell cycle protein. The t(11;14) can be reliable detected by fluorescence in situ hybridization (FISH), and cyclinD1 by immunohistochemistry. These 2 tests are included in the standard panel for definitive diagnosis in conjunction with routine histology, immunohistochemistry and immunophenotypic flow studies. Clinically, mantle cell lymphoma affects more male than female (ratio 3:1), and the mean age at diagnosis is around 68 years. Most MCL patients present with advanced stage disease (stage 3 & 4), and up to 25% can present with extranodal disease which typically involves gastrointestinal tract.
Mantle cell lymphoma must be differentiated from other subtypes of non-Hodgkin’s lymphoma which are composed of small to medium sized cells, including chronic lymphocytic leukemia (CLL/SLL), follicular lymphoma (FL) and marginal zone lymphoma (MZL). In contrast to MCL B-cells which are positive for CD5, negative for CD23, and express cyclin D1, CLL cells are positive for both CD5 and CD23, but negative for cyclin D1, while FL and MZL cells are generally negative for CD5 and do not express cyclin D1. Since the clinical management for MCL can be different from other NHL subtypes, I would like to emphasize the importance for patients with newly diagnosed MCL to get a second opinion / confirmation from a lymphoma center with expertise in hematopathology.
There is a wide range of clinical behaviors for mantle cell lymphoma. Some patients can present with very indolent disease despite having advanced stage, and can be managed by conservative observation without cytotoxic treatment initially. Others may present with rapidly progressive disease that requires immediate systemic chemotherapy. Unlike certain kind of aggressive lymphoma, such as diffuse large B-cell lymphoma or Hodgkin’s lymphoma, which can be cured in a majority of patients, mantle cell lymphoma remains incurable. Therefore, individualized approaches to maximize survival and maintain quality of life are important treatment goals.
Mantle cell lymphoma must be differentiated from other subtypes of non-Hodgkin’s lymphoma which are composed of small to medium sized cells, including chronic lymphocytic leukemia (CLL/SLL), follicular lymphoma (FL) and marginal zone lymphoma (MZL). In contrast to MCL B-cells which are positive for CD5, negative for CD23, and express cyclin D1, CLL cells are positive for both CD5 and CD23, but negative for cyclin D1, while FL and MZL cells are generally negative for CD5 and do not express cyclin D1. Since the clinical management for MCL can be different from other NHL subtypes, I would like to emphasize the importance for patients with newly diagnosed MCL to get a second opinion / confirmation from a lymphoma center with expertise in hematopathology.
There is a wide range of clinical behaviors for mantle cell lymphoma. Some patients can present with very indolent disease despite having advanced stage, and can be managed by conservative observation without cytotoxic treatment initially. Others may present with rapidly progressive disease that requires immediate systemic chemotherapy. Unlike certain kind of aggressive lymphoma, such as diffuse large B-cell lymphoma or Hodgkin’s lymphoma, which can be cured in a majority of patients, mantle cell lymphoma remains incurable. Therefore, individualized approaches to maximize survival and maintain quality of life are important treatment goals.
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