Book contents
- Frontmatter
- Contents
- List of contributors
- Preface
- Part I LYMPHOMA OVERVIEW
- Part II LYMPHOMA SUBTYPES
- 7 Hodgkin's lymphoma
- 8 Follicular lymphoma
- 9 MALT lymphoma and other marginal zone lymphomas
- 10 Small lymphocytic lymphoma and its variants
- 11 Mantle cell lymphoma
- 12 Diffuse large B-cell lymphoma
- 13 Burkitt's and lymphoblastic lymphomas
- 14 Central nervous system lymphoma
- 15 T-cell lymphoma
- 16 Cutaneous lymphoma
- 17 Lymphoma in the immunosuppressed
- Index
- References
11 - Mantle cell lymphoma
from Part II - LYMPHOMA SUBTYPES
Published online by Cambridge University Press: 05 March 2010
- Frontmatter
- Contents
- List of contributors
- Preface
- Part I LYMPHOMA OVERVIEW
- Part II LYMPHOMA SUBTYPES
- 7 Hodgkin's lymphoma
- 8 Follicular lymphoma
- 9 MALT lymphoma and other marginal zone lymphomas
- 10 Small lymphocytic lymphoma and its variants
- 11 Mantle cell lymphoma
- 12 Diffuse large B-cell lymphoma
- 13 Burkitt's and lymphoblastic lymphomas
- 14 Central nervous system lymphoma
- 15 T-cell lymphoma
- 16 Cutaneous lymphoma
- 17 Lymphoma in the immunosuppressed
- Index
- References
Summary
INTRODUCTION
Mantle cell lymphoma (MCL) is a unique subtype of non-Hodgkin's lymphoma (NHL) characterized in almost all cases by the chromosomal translocation t(11;14)(q13;q32) and nuclear cyclin D1 overexpression. Most patients present with advanced-stage disease, often with extranodal dissemination, and typically pursue an aggressive clinical course, with median survival historically averaging 3–4 years. No standard curative therapy exists, even with intensive induction regimens followed by autologous stem-cell transplantation. However, a number of recent insights into the molecular and cellular biology of the disease, as well as combined immunochemotherapy and novel therapeutic approaches, hold promise for improved outcomes. Importantly, MCL provides a paradigm for therapeutic targeting in neoplasms with dysregulated cell cycle and apoptotic pathways.
MCL comprises approximately 4–8% of all NHL, with a preponderance of older males relative to other lymphoma subtypes. The male-to-female ratio is 2–3 : 1 and median age at presentation is 60–65 years. No specific etiologic factors have been identified for this disease. An increased risk of lymphoid neoplasms has been reported in first-degree relatives of MCL patients, although MCL occurrence among multiple family members appears to be quite rare.
CLINICAL PRESENTATION
MCL typically presents in advanced stage; over 90% of patients are stage III–IV at diagnosis, frequently with B symptoms. Splenomegaly is seen in half or more of patients, often in association with a leukemic phase.
- Type
- Chapter
- Information
- Lymphoma: Pathology, Diagnosis and Treatment , pp. 154 - 167Publisher: Cambridge University PressPrint publication year: 2007
References
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