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Mantle cell lymphoma (MCL) is a subtype of non-Hodgkin lymphoma that is characterized by widespread disease in most patients at the time of diagnosis. The lymphoma cells usually contain a translocation between chromosomes 11 and 14, juxtaposing the gene encoding the immunoglobulin heavy chain and the gene encoding cyclin D1. The cells overexpress cyclin D1, which can be identified with immunocytochemistry, providing a relatively easy marker for diagnosis. The disease has a lower rate of complete remission, duration of response, and overall survival after conventional chemotherapy when compared with other lymphomas. More aggressive, multidrug therapy, usually including cytarabine and rituximab, has improved outcome, and the median survival has increased to about 5 years. Consolidation therapy with autologous stem cell transplantation is also used. New chemotherapeutic agents and radioimmunotherapy may add further to advances in treatment.

Acronyms and Abbreviations

Acronyms and abbreviations that appear in this chapter include: ATM, ataxia-telangiectasia mutation; bcl-1, b-cell lymphoma 1; CDK, cyclin D kinase; CHOP, cyclophosphamide, doxorubicin, vincristine, prednisone; CLL, chronic lymphocytic leukemia; CR, complete response; Cru, complete response unconfirmed; DFS, disease-free survival; DHAP, dexamethasone, high-dose cytarabine, cisplatin; E2F, elongation factor 2; EFS, event-free survival; FFS, failure-free survival; LDH, lactate dehydrogenase; MCL, mantle cell lymphoma; MIPI, mantle cell international prognostic index; mRNA, messenger RNA; mTOR, mammalian target of rapamycin; NF-κB, nuclear factor-κB; ORR, objective response rate; OS, overall survival; PFS, progression-free survival; PI3K, phosphoinositol 3 kinase; RB1, retinoblastoma 1; R-HDS, rituximab high-dose sequential therapy; SCT, stem cell transplantation; SLL, small lymphocytic leukemia; TTF, time to treatment failure.

Mantle cell lymphoma (MCL) is a lymphoma subtype that usually is characterized by cells carrying an immunophenotype similar to lymphocytes in the mantle zone of normal germinal follicles, secretory immunoglobulin (sIg) M+, sIgD+, CD5+, CD20+, CD10–, CD43+, and the cytogenetic abnormality t(11;14) (q13;q32) in the tumor cells, resulting in the overexpression of cyclin D1. MCL had been previously named intermediate lymphocytic lymphoma, centrocytic lymphoma, lymphocytic lymphomas with intermediate differentiation, and marginal zone lymphoma. In 1992, a proposal was made to replace the previous designations by the term “mantle cell lymphoma” because of the morphologic and immunophenotypic similarity of the tumor cells to the lymphocytes found in the mantle zone of secondary germinal centers and the resting B lymphocytes of primary germinal centers.1 In 1994, the term mantle cell lymphoma was incorporated into the revised European-American classification of the International Lymphoma Study Group.2 MCL remains a distinctive subtype of lymphoma in the World Health Organization classification of malignant lymphopoietic disorders.3

MCL was thought to represents approximately 6 percent of all non-Hodgkin lymphomas,4 although a lower incidence was reported in a recent analysis.5 In the latter report, MCL represented approximately 3 percent of all lymphoma cases reported in the United States between 1992 and 2004. The overall annual incidence rate was 0.55 cases per 100,000 persons. Between 1992 and 2004, the age-adjusted annual incidence more than doubled ...

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