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MANAGEMENT OF MANTLE CELL LYMPHOMA IN TUNISIA

MANAGEMENT OF MANTLE CELL LYMPHOMA IN TUNISIA. R BEN LAKHAL , L KAMMOUN , K ZAHRA , S KEFI. Sousse 25 MAY 2012. MCL uncommon lymphoma. T lymphoblastic: 2%. Other: 9%. Marginal zone, nodal: 2%. Burkitt: 2%. Anaplastic large cell: 2 %. Diffuse large B cell: 31%.

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MANAGEMENT OF MANTLE CELL LYMPHOMA IN TUNISIA

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  1. MANAGEMENT OF MANTLE CELL LYMPHOMA IN TUNISIA R BEN LAKHAL , L KAMMOUN , K ZAHRA , S KEFI Sousse 25 MAY 2012

  2. MCL uncommon lymphoma T lymphoblastic: 2% Other: 9% Marginal zone, nodal: 2% Burkitt: 2% Anaplastic large cell: 2% Diffuse large B cell: 31% Mediastinal large B cell: 2% Mantle cell: 6% Small lymphocytic/CLL: 7% Peripheral T cell: 7% Follicular: 22% Marginal zone, extranodal: 8% Armitage JO, et al. J Clin Oncol. 1998;16:2780-2795.

  3. 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.

  4. 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

  5. 32 patients : 2000-2011 3 centers : Tunis : 20 patients Sousse : 7 patients Sfax : 5 patients PATIENTS

  6. Epidémiologic Features Annual incidence of MCL

  7. Epidémiologic Features Median age : 62 years ( 30-84 years) 23 males ( 72%) Sex-ratio : 2.55

  8. Clinical and biological features

  9. Prognosis of patients

  10. Methods 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

  11. Treatment Features • 30 patients treated (1death, 1 lost to follow-up) • 25/30 patients received Rituximab (83%) • 2 patients treated on 2001 • 3 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

  12. Response of Treatment 30 patients treated 4 lost to follow-up (13 %) 26 evaluables patients CR 11 patients (42%) Failure/progression 08 patients (31%) PR 07 patients (27%) ORR = 69% 6 Deaths (4 toxic deaths)

  13. Response according to prognostic factors

  14. Response according to prognostic factors

  15. OVERALL SURVIVAL (OS) OS : 60% (5years)

  16. OS according to prognostic factors • One significant adverse prognostic factor : failure to treatement OR Failure

  17. Event free survival (EFS) EFS(5years) : 52%

  18. DEATHS : 8 patients • 1 death before treatement • 5 toxic deaths • 2 deaths progression RELAPSES • One relapse • Late relapse (5 years) • Post ASCT

  19. DISCUSSION • Epidemiological , clinical and biological characteristics of Tunisian patients are comparables to littérature data. • annual incidence increasing ? or improvement of diagnosis tools ?

  20. DISCUSSION PROGNOSIS • 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%)

  21. MIPI > FLIPI > IPI

  22. Biologic MIPI = MIPI + proliferation marker Ki-67

  23. DISCUSSION TREATMENT • 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%

  24. Role of cytarabine (Ara-C)

  25. Role of ASCT

  26. 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

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