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The Leukemia/Bone Marrow Transplant Program of BC

Healthcare Professionals
Cancer Management Guidelines

Mantel Cell Lymphoma

Updated: March 2011 - currently under review

Diagnosis

Mantle cell lymphoma comprises approximately 5% of all non-Hodgkin’s lymphoma. It is a B-cell lymphoma which has a characteristic translocation between chromosome 11 and 14. This translocation results in deregulated overexpression cyclin D1. Patients who are diagnosed with this lymphoma should be referred to a local oncologist/hematologist to initiate treatment. The local oncologist/hematologist should then consider referring the patient to the Leukemia/BMT Program at the Vancouver General Hospital for possible high-dose chemotherapy followed by stem cell rescue as consolidation of their initial chemotherapy (autotransplant). The referral to the BMT Program should be made early in order to have adequate time to arrange for the autotransplant. 

Required Tests

  • Lymph node / tissue biopsy
  • CBC and differential
  • Electrolytes, BUN, creatinine, uric acid, liver function tests, LDH
  • INR, PTT and fibrinogen
  • Bone marrow aspirate and biopsy with cytogenetics analysis and flow cytometry for immunophenotyping
  • Computed tomography (CT) scan of the neck, chest, abdomen and pelvis. CT of the head should also be considered, especially if there are CNS symptoms.

Staging

The stage of disease is of major therapeutic and prognostic significance. The staging system used is based on the Ann Arbor system with additional consideration of the bulk or size of individual tumours. The formal stage is assigned using the following system. 

 Stage

Involvement 

1

Single lymph node region (1) or one extralymphatic site (1E) 

2

Two or more lymph node regions, same side of the diaphragm (s) or local extralymphatic extension plus one or more lymph node regions, same side of the diaphragm (2E)

3

Lymph node regions on both sides of diaphragm (3) which may be accompanied by local extralymphatic extension (3E)

4

Diffused involvement of one or more extralymphatic organs or sites

Symptoms

 A =  

No B symptoms 

 B = 

Presence of at least one of these:

  1. Unexplained weight loss > 10% baseline during 6 months prior to staging
  2. Unexplained fever > 38°C
  3. Night sweats

Prognosis

Mantle Cell lymphoma remains an incurable lymphoma. For patients who are eligible for autotransplant, median survival is greater than 5 years.

Treatment

Algorithms

Mantel Cell Treatment Algorithm

Protocols

BCCA Chemotherapy Protocols and PPOs

Publications

  1. Mangel J, Leitch HA, Connors JM, Buckstein R, Imrie K, Spaner D, Crump D, Crump M, Pennell N, Boudreau A & Berinstein NL. Intensive chemotherapy and autologous stem-cell transplantation plus rituximab is superior to conventional chemotherapy for newly diagnosed advanced stage mantle-cell lymphoma: a matched pair analysis. Ann Oncol 15:283-290, 2004.

 

The information contained in these guidelines is a statement of consensus of Leukemia/BMT Program of BC professionals regarding their views of currently accepted approaches to treatment. Any clinician seeking to apply or consult these documents is expected to use independent medical judgment in the context of individual clinical circumstances to determine any patientís care or treatment. Use of these guidelines and documents is at your own risk and is subject to the Leukemia/BMT Program of BCís terms of use available at Terms of Use.

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