eMedicine Specialties > Hematology > Stem Cells and Disorders

Lymphoma, Mantle Cell

Author: Muhammad Rashid Abbasi, MD, Assistant Professor of Medicine, Albert Einstein College of Medicine; Consulting Staff, Department of Internal Medicine, Division of Hematology/Oncology, Jacobi Medical Center, Morristown Memorial Hospital, and St Clare's Hospital
Coauthor(s): Joseph A Sparano, MD, Professor of Medicine, Albert Einstein College of Medicine/Cancer Center; Program Director, Director of Breast Medical Oncology, Department of Internal Medicine, Division of Oncology, Montefiore Medical Center
Contributor Information and Disclosures

Updated: Feb 22, 2010

Introduction

Background

Mantle cell lymphoma (MCL) is recognized in the Revised European-American Lymphoma and World Health Organization classifications as a distinct clinicopathologic entity. MCL was not recognized by previous lymphoma classification schemes; it was frequently categorized as diffuse small-cleaved cell lymphoma (by the International Working Formulation) or centrocytic lymphoma (by the Kiel classification). In the International Lymphoma Classification Project, it accounted for 8% of all non-Hodgkin lymphomas (NHLs).

Pathophysiology

MCL is a lymphoproliferative disorder derived from a subset of naive pregerminal center cells localized in primary follicles or in the mantle region of secondary follicles. Most cases of MCL are associated with chromosome translocation t(11;14)(q13;q32). This translocation involves the immunoglobulin heavy-chain gene on chromosome 14 and the BCL1 locus on chromosome 11. The molecular consequence of translocation is overexpression of the protein cyclin D1 (coded by the PRAD1 gene located close to the breakpoint). Cyclin D1 plays a key role in cell cycle regulation and progression of cells from G1 phase to S phase by activation of cyclin-dependent kinases.

Frequency

United States

NHL represents approximately 4% of all cancer diagnoses and is the seventh most common cancer. MCL represents 2-10% of all NHLs. In 1999, more than 55,000 new cases of NHL were diagnosed. The incidence of NHL of all types has increased by approximately 40% over the last 20 years, although the cause for this increase is unknown.

International

NHLs are 5 times more common than Hodgkin disease, representing approximately 4% of all cancers diagnosed internationally. The exact international prevalence of MCL is difficult to estimate because of the lack of uniform classification and procedures used for diagnosis.

Mortality/Morbidity

MCL is associated with a poor prognosis. Although MCL represents only 6% of NHLs, it remains incurable with current chemotherapeutic approaches. Despite response rates of 50-70% with many regimens, the disease typically progresses after chemotherapy. The median survival time is approximately 3 years (range, 2-5 y); the 10-year survival rate is only 5-10%.

  • Developing reliable prognostic markers has been difficult in the absence of better-standardized therapy.
  • The blastoid variant, commonly associated with TP53 mutations, has been associated with a worse prognosis. Gene expression profile analysis identified MCL patient subsets with more than 5 years' difference in median survival, based on cyclin D1 and other proliferation signature genes.

Race

Overall, whites are at higher risk for developing NHLs than blacks and Asian Americans.

Sex

The male-to-female ratio is 4:1.

Age

The age range at presentation is 35-85 years. Median age is 60 years.

Clinical

History

  • Stage IV disease in 70% of patients
  • B symptoms, which include fever, night sweats, and weight loss, in 40% of patients
  • Generalized lymphadenopathy
  • Abdominal distension from hepatosplenomegaly
  • Fatigue from anemia or bulky disease
  • Less common symptoms caused by extranodal involvement of GI tract, lungs, and CNS

Physical

  • Generalized lymphadenopathy in 90%
  • Splenomegaly in 60% (may be massive)
  • Hepatomegaly in 30%
  • Poor performance status in 20%
  • Less commonly, palpable masses in skin, breast, and salivary glands

Causes

  • No causative factor has been identified for MCL or for most patients with NHL of other types.
  • NHL has been associated with viral infection (Ebstein-Barr virus, HIV, human T-lymphotropic virus type 1, human herpesvirus 6), environmental factors (pesticides, hair dyes), and primary and secondary immunodeficiency.
  • Nonrandom chromosomal and molecular rearrangements play a major role in the pathogenesis of many lymphomas. The association of t(11;14)(q13;q32) with MCL suggests a causative role.

More on Lymphoma, Mantle Cell

Overview: Lymphoma, Mantle Cell
Differential Diagnoses & Workup: Lymphoma, Mantle Cell
Treatment & Medication: Lymphoma, Mantle Cell
Follow-up: Lymphoma, Mantle Cell
References
Further Reading
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References

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Further Reading

Related eMedicine Topics

Clinical Trials

Clinical Guidelines

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Keywords

mantle cell lymphoma, MCL, non-Hodgkin lymphoma, NHL, lymphocytic lymphoma of intermediate differentiation, intermediate lymphocytic lymphoma, ILL, diffuse poorly differentiated lymphocytic lymphoma, PDL, centrocytic small-cell lymphoma, diffuse small-cleaved cell lymphoma, DSCCL, mantle zone lymphoma, diffuse intermediate differentiated lymphocytic lymphoma

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Contributor Information and Disclosures

Author

Muhammad Rashid Abbasi, MD, Assistant Professor of Medicine, Albert Einstein College of Medicine; Consulting Staff, Department of Internal Medicine, Division of Hematology/Oncology, Jacobi Medical Center, Morristown Memorial Hospital, and St Clare's Hospital
Muhammad Rashid Abbasi, MD is a member of the following medical societies: American College of Physicians, American Medical Association, and American Society of Hematology
Disclosure: Nothing to disclose.

Coauthor(s)

Joseph A Sparano, MD, Professor of Medicine, Albert Einstein College of Medicine/Cancer Center; Program Director, Director of Breast Medical Oncology, Department of Internal Medicine, Division of Oncology, Montefiore Medical Center
Joseph A Sparano, MD is a member of the following medical societies: American Association for Cancer Research, American College of Physicians, and American Society of Hematology
Disclosure: Nothing to disclose.

Medical Editor

Michael Paul Kosty, MD, Associate Director, Associate Professor, Department of Internal Medicine, Divisions of Supportive Care Services and Hematology and Oncology, Ida M and Cecil H Green Cancer Center, Scripps Clinic
Michael Paul Kosty, MD is a member of the following medical societies: American College of Physicians, American Society of Hematology, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Wendy Hu, MD, Consulting Staff, Department of Hematology/Oncology and Bone Marrow Transplantation, Huntington Memorial Medical Center
Wendy Hu, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Physicians, American Society for Blood and Marrow Transplantation, American Society of Hematology, and Physicians for Social Responsibility
Disclosure: Nothing to disclose.

CME Editor

Rajalaxmi McKenna, MD, FACP, Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Clinical Oncology, American Society of Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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