MANAGEMENT OF MANTLE CELL LYMPHOMA IN TUNISIA R BEN LAKHAL, L KAMMOUN, K ZAHRA, S KEFI Sousse 25 MAY...
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Transcript of MANAGEMENT OF MANTLE CELL LYMPHOMA IN TUNISIA R BEN LAKHAL, L KAMMOUN, K ZAHRA, S KEFI Sousse 25 MAY...
MANAGEMENT OF MANTLE CELL LYMPHOMA IN TUNISIA
R BEN LAKHAL , L KAMMOUN , K ZAHRA , S KEFI
Sousse 25 MAY 2012
MCL uncommon lymphoma
Armitage JO, et al. J Clin Oncol. 1998;16:2780-2795.
Diffuse large B cell: 31%
Follicular: 22%Marginal zone, extranodal: 8%
Peripheral T cell: 7%
Small lymphocytic/CLL: 7%
Mantle cell: 6%
Mediastinal large B cell: 2%
Anaplastic large cell: 2%
Burkitt: 2%
Marginal zone, nodal: 2%
T lymphoblastic: 2%
Other: 9%
Introduction
Mantel Cell Lymphoma (MCL) :
Aggressive B-cell Malignancy.
Complex pathophysiology : t(11, 14) aberrant
expression of cylcin D1.
Advanced non-bulky disease.
Diagnosed at age 60 to 65 years.
Short median survival (3 years) despite intensive
therapy.
OBJECTIVE
Retrospective Tunisian multicenter study :
Analyze epidemiological,clinical and biological features of tunisian MCL patients.
Evaluate the response to treatement according to classical prognostic factors.
Analyze the event free survival (EFS) and the overal survival (OS) according to prognostic factors
32 patients : 2000-2011
3 centers :
Tunis : 20 patients
Sousse : 7 patients
Sfax : 5 patients
PATIENTS
Epidémiologic Features
0
1
2
3
4
5
6
7
8
9
2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011
Annual incidence of MCL
Epidémiologic Features Median age : 62 years ( 30-84 years)
23 males ( 72%) Sex-ratio : 2.55
Clinical and biological features
N Pencentage
B symptoms
No 20 62.5%
Yes 12 37.5%
PS
2 23 72%
>2 9 28%
Stage
Early 5 18%
Advanced 27 84%
LDH
>Nle 21 66%
<Nle 11 34%
Bone Marrow involvement
Yes 19 60%
No 13 40%
Prognosis of patients
N Percentage
IPI
0-1 12 38
2-3 20 62
MIPI (16 cases)
Low risk 3 19
Intermediate 5 31
High risk 8 50
Methods
STATISTIC STUDY STATISTIC STUDY
1- Predictives response factors :
( Chi-square test, p < 0.05 )
2 - The EFS «event free survival» and the OS «overall survival» :
(Kaplan-Meier method and Log-Rank test)
- Univariate study
- Multivariate study
Treatment Features
30 patients treated (1death, 1 lost to follow-up) 25/30 patients received Rituximab (83%)
2 patients treated on 20013 patients > 75 years (Mini-CEOP)
Chemotherapy : CHOP/DHAP = 12 patients ( 40%) CHOP = 13 patients ( 43.3%)Velcade – CHP = 2 patients (6.6%) Mini-CEOP = 3 patients ( 10%)
Autologous stem cell transplantation = 5 patients (13pts<60 years)
Allogeneic stem cell transplantation : 1 patient
Response of Treatment
30 patients treated
4 lost to follow-up (13 %)
26 evaluables patients
CR11 patients
(42%)
PR07 patients
(27%)
Failure/progression 08 patients
(31%)
ORR = 69% 6 Deaths (4 toxic deaths)
Gender : ORR P
Male 68.4% 0.88 NS
Female 71.4%
Stage :
Early 100% 0.13 NS
Advanced 61.9%
LDH:
N 72.7% 0.94 NS
>N 71.4%
PS :
2 70% 0.84 NS
>2 75%
Response according to prognostic factors
ORR P
IPI :
0-1 72.7% 0.85 NS
2-3 69.2%
Auto :
Yes 100% 0.2 NS
No 65%
Rituximab :
Yes 72.7% 0.37 NS
No 50%
DHAP:
Yes 81.8% 0.23 NS
No 60%
Response according to prognostic factors
OVERALL SURVIVAL (OS)
Recul en mois
13212010896847260483624120
Surv
ie g
loba
le
1,0
,9
,8
,7
,6
,5
,4
,3
,2
,1
0,0
OS : 60% (5years)
OS according to prognostic factors
One significant adverse
prognostic factor :
failure to treatement
Recul en mois
13212010896847260483624120
Surv
ie g
loba
le
1,0
,9
,8
,7
,6
,5
,4
,3
,2
,1
0,0
Réponse
3+4+5
0+1+2
p < 0,001
OR
Failure
Event free survival (EFS)
Recul en mois
12010896847260483624120
Su
rvie
sans
év
én
em
en
t
1,0
,9
,8
,7
,6
,5
,4
,3
,2
,1
0,0
EFS(5years) : 52%
DEATHS : 8 patients
1 death before treatement 5 toxic deaths 2 deaths progression
RELAPSES
One relapse Late relapse (5 years) Post ASCT
DISCUSSION
Epidemiological , clinical and biological characteristics of Tunisian patients are comparables to littérature data.
annual incidence increasing ?
or
improvement of diagnosis tools ?
DISCUSSION
There is no generally established prognostic
index for patients with MCL.
For our patients :
IPI>2 (High risk patients) : 20 (62%)
MIPI evaluated in 16 patients
High risk patients : 8 (50%)
PROGNOSIS
MIPI > FLIPI > IPI
Biologic MIPI = MIPI +
proliferation marker Ki-67
DISCUSSION
Aggressive therapies including chemo-immunotherapy or high dose chemotherapy followed by autologous stem cell transplant have been shown to improve outcome BUT
no standard therapy offers the potential for cure.
Our patients :
Immunotherapy : all younger patients
RCHOP/RDHAP : 12 patients
ASCT : only 5 patients (13 pts < 60 yrs)
ORR : 69% (DHAP : ORR at 80%)
OS : 60%
EFS : 52%
TREATMENT
Role of cytarabine (Ara-C)
Role of ASCT
CONCLUSION Epidemiological , clinical and biological characteristics of Tunisian
patients are comparables to littérature data.
Therapeutic results must be improved +++
Younger patients (< 60 yrs) : HD Arac + ASCT
Patients <40yrs : allogeneic transplantation
Older patients : RCHOP +/- Rituximab maintenance
Salvage therapy +++
Better management of toxicity