Guidelines on Diagnosis and Treatment of Malignant Lymphomas

Prognostic factors/ index
The IPI is generally used, however this has been modified for
advanced MCL to the MIPI, which has not gained widespread
popularity because of the complex statistics needed to score
patients based on age, ECOG score, LDH and leucocyte count
(Blood 2008,111:558-565)
IPI Score
Potential pitfalls
a. Confusion with other lymphomas notably FL and CLL/SLL
b. Blastic MCL may be mis-diagnosed
OS at 5 years
0 23%
1 45%
2 54%
3 25%
4 23%
5 0%
c. Failure to recognise multiple lymphomatous polyposis
Treatment
There are no randomised controlled trials defining optimal first
line treatment. The best published outcome is with R-HCVAD
which gave a CR rate of 90% and a progression-free survival
(PFS) of 75% at 5 years. This should be considered for younger
patients and possibly all patients with a good performance
status. Regimens containing anthracyclines in historical series
offered no advantage over those without an anthracycline.
The Nordic group has obtained equivalent outcomes using
intensified CHOP with additional cytosine arabinoside followed
by a first remission autograft.
Rituximab with fludarabine, cyclophosphamide and mitoxantrone
(FCMR or FCM) may be effective for those unable to tolerate R-
HCVAD. Proteasome inhibitors are a biologically logical treatment
and in Phase II studies show encouraging results.
MCL is considered incurable with conventional-dose
chemotherapy. It is appropriate therefore to consider allogeneic
transplant for those who achieve a CR or good PR, are fit and
have an HLA compatible sibling donor. If a sibling donor is not
available then high-dose chemotherapy with autologous stem cell
support should be considered in first complete remission. Results
for those given autologous transplantation in partial remission or
in complete remission at a later stage in their disease are poor
(OS< 20% at 5 years).
Response Evaluation and Follow up
There are no agreed recommendations for the evaluation and
follow-up of patients with MCL, but the recommendations for
FL can be applied. Approaches may need to be revised as
treatments and treatment outcomes improve.
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