Mantle Cell Lymphoma (MCL)

This has a variable clinical course from indolent to aggressive. Patients usually present with lymphadenopathy but blood and marrow involvement may be present at diagnosis or develop during the course of the disease.

In the blood, cells range between lymphocytes and immature lymphoid cells with some nuclear and cytoplasmic irregularity. There are blastic variants. Immunophenotype usually shows CD20+, 22+, 23-, sIg+. Lambda light chain restriction predominates. The cells are cyclin D1+ in contrast to CLL.

Most cases have the t(11;14) translocation causing upregulation of BCL1. Changes in p27 and p53 oncogenes identify a subtype with a poorer prognosis.

Note: For a further discussion of the distinguishing laboratory features of some indolent NHL’s, e.g. CLL/SLL, follicular lymphoma, marginal zone lymphoma, mantle cell lymphoma and hairy cell leukaemia, see ASH EPB 2004 p.229 Table 5. This describes the laboratory differential diagnosis of these conditions.

In This Section

Management

This presents problems as many patients are elderly and MCL often pursues a rapidly progressive course.

The outcome in this disease depends much more on the prognostic index of the MCL rather than any treatment selected.

Some patients have an indolent form of the disease, typically non-nodal, with splenomegaly and blood involvement. A watch and wait policy may be justified in these cases.

The choice of chemotherapy should be individualised. For:

younger and fitter patients, include Nordic protocol and autologous transplant.
older and fitter patients, include rituximab bendamustine, and high dose cytarabine – perhaps with transplant.
frailer patients, include less intense therapy – perhaps RB or RCVP.

Ibrutinib is the first choice internationally for relapsed patients, but is not funded in New Zealand.

See Martin, P. (2017). "Optimizing therapy for mantle cell lymphoma." Hematology Am Soc Hematol Educ Program 2017(1): 304-309.

Further Reading


About this Canterbury DHB document (5958):
Document Owner:	Peter Ganly (see Who's Who)
Last Reviewed:	December 2021
Next Review:	December 2024
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Note: Only the electronic version is controlled. Once printed, this is no longer a controlled document. Disclaimer The Haematology Department Protocols and Guidelines (the Red Book) are intended as a guide for registered health professionals to communicate guidelines and share best practices with supporting health professionals within New Zealand district health boards. They are not intended to be provided to or used as a reference for patients or non-registered health professionals. All health professionals must exercise their own clinical judgement and use pertinent clinical data when treating patients. The authors and editors of this publication have checked with sources believed to be reliable in their effort to provide information that is complete and in accord with the standards accepted at the time of publication. However, in view of the possibility of human error or changes in medical science, neither the authors, editors, publisher, nor any other party who has been involved in preparing or publishing this work warrants that the information contained in it is accurate or complete in every respect. They therefore have no liability (whether in tort (including negligence) or otherwise) for any loss or damage arising out of any reliance on this information. This publication has been compiled by the staff and contractors of Canterbury District Health Board (CDHB) and as such CDHB has copyright for this book. Amendments must be authorised in accordance with the documented process. No release of part of, or all of, this publication to any other party or organisation is permissible without the prior approval of the designated document owner.
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Topic Code: 5958