eMedicine Specialties > Hematology > Stem Cells and Disorders

Lymphoma, Mantle Cell: Differential Diagnoses & Workup

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: May 14, 2007

Differential Diagnoses

Chronic Lymphocytic Leukemia
Hairy Cell Leukemia
Lymphoma, Diffuse Large Cell
Lymphoma, Follicular
Lymphoma, Non-Hodgkin
Myeloproliferative Disease

Other Problems to Be Considered

Follicular center cell lymphoma
Lymphoplasmacytic lymphoma
Marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue type
Nonneoplastic hyperplasias (Castleman disease, mantle zone hyperplasia, reactive follicular hyperplasia)
Small lymphocytic lymphoma

Workup

Laboratory Studies

  • CBC count
    • Anemia and cytopenias are secondary to bone marrow infiltration.
    • Lymphocytosis of more than 4000/µL occurs in 20-40% of cases.
  • Chemistry
    • Patients have elevated lactate dehydrogenase levels, which correlate with tumor burden.
    • Patients have abnormal liver function test results from liver involvement.
  • Beta2-microglobulin: The level may be elevated, which indicates a poor prognosis.
  • Rare findings: These include hypogammaglobulinemia, monoclonal gammopathy, and a positive Coombs test result.

Imaging Studies

  • Body CT scan: This study is important for initial staging and for assessing the patient's response to treatment.

Other Tests

Immunocytochemistry

Tumor cells are monoclonal B cells that express surface immunoglobulin, immunoglobulin M, or immunoglobulin D. Cells are characteristically CD5+ and pan B-cell antigen positive (eg, CD19, CD20, CD22) but lack expression of CD10 and CD23. Cyclin D1 is overexpressed. Immunophenotyping helps differentiate MCL from other small B-cell lymphomas (see Table).1,2

Differential Diagnosis of MCL by Immunophenotyping

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Table
DiseaseCD5CD20CD23CD10CD103FMC7Cyelin
D1		Sig*
MCL+++–––+/–++
B-CLL/SLL† +++––––+
PLL‡ –/++++/–––+–++
MZL§ –++–––+/––+
SLVL|| –++–/+––+/––++
LPL –+––––/+–++
FL# –++–+–+/––++
HCL**–+––++–/+++
DiseaseCD5CD20CD23CD10CD103FMC7Cyelin
D1		Sig*
MCL+++–––+/–++
B-CLL/SLL† +++––––+
PLL‡ –/++++/–––+–++
MZL§ –++–––+/––+
SLVL|| –++–/+––+/––++
LPL –+––––/+–++
FL# –++–+–+/––++
HCL**–+––++–/+++

*Surface immunoglobulins.
† B-cell chronic lymphocytic leukemia/small lymphocytic leukemia.
‡ Prolymphocytic leukemia.
§ Marginal zone leukemia.
|| Splenic lymphoma with villous lymphocytes.
Lymphoplasmacytic lymphoma.
# Follicular lymphoma.
**Hairy cell leukemia.

Cytogenetics
Most cases of MCL are associated with a chromosome translocation between chromosome 11 and 14, t(11;14)(q13;q32).3

Procedures

  • Perform lymph node biopsy and aspiration together because aspiration alone is insufficient to establish a diagnosis.
  • Use bone marrow aspirate/biopsy results for staging rather than diagnostic purposes.

Histologic Findings

Lymph node

Tumor is characterized by expansion of the mantle zone that surrounds the lymph node germinal centers by small-to-medium atypical lymphocytes. These cells have irregular and indented nuclei, moderately coarse chromatin, and scant cytoplasm, resembling smaller cells of follicular lymphoma. However, mitoses are more numerous and large cells are infrequent. A nodular appearance may be evident from expansion of the mantle zone in 30-50% of patients early in the disease. As disease progresses, the germinal centers become effaced, with obliteration of lymph node architecture.

A blastic variant of MCL, demonstrating numerous medium-to-large blastlike cells, has been reported and is associated with a more aggressive clinical course.

Bone marrow

In bone marrow sections, neoplastic cells may infiltrate in a focal, often paratrabecular or diffuse pattern. Diagnosis of MCL should not be based on the examination of bone marrow alone; obtaining a lymph node biopsy is required.

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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Keywords

mantle cell lymphoma, MCL, lymphocytic lymphoma of intermediate differentiation, intermediate lymphocytic lymphoma, ILL, diffuse poorly differentiated lymphocytic lymphoma, PDL, centrocytic lymphoma, diffuse small-cleaved cell lymphoma, DSCCL, mental zone 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 Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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