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.
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.
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.
Single lymph node region (1) or one extralymphatic site (1E)
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)
Lymph node regions on both sides of diaphragm (3) which may be accompanied by local extralymphatic extension (3E)
Diffused involvement of one or more extralymphatic organs or sites
No B symptoms
Presence of at least one of these:
Unexplained weight loss > 10% baseline during 6 months prior to staging
Unexplained fever > 38°C
Mantle Cell lymphoma remains an incurable lymphoma. For patients who are eligible for autotransplant, median survival is greater than 5 years.
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.