Abstract B158: Clinical features and management of mantle cell lymphoma

Abstract

Abstract Introduction: Mantle cell lymphoma (MCL) is a relatively uncommon subtype of lymphoid malignancy and represents 3%–10% of malignant lymphoma, and now appears as a biologic and therapeutic model in the understanding and treatment of hematologic malignancies. Diagnostic procedures include histomorphology, immunophenotype (CD5+, CD19/20+), Ki-67 staining and mandatory detection of cyclin D1 overexpression or t(11;14)(q13;q32). The treatment is very heterogenous and the evolution is marked by numerous relapses. Patients and Methods: During the period from January 2012 to January 2017, we conducted a retrospective and prospective study on the epidemiologic, clinical, prognostic and therapeutic profile of mantle lymphoma. This mono-centric study focused on 20 cases managed in the hematology department of the Annaba University Hospital. The diagnostic method was based on lymph node biopsy and immunophenotyping. The clinical characteristics (according to the international prognostic index of MCL [MIPI]), anatomopathologic (Ki67, blastoid appearance) and radiologic were collected, as well as the different treatment lines were reported. It should be noted that no patient has benefited from a cytogenetic study in search of t (11; 14). Results: Mean age was 63.5 years (range 44- 87). There was a predominance of male subjects with a sex ratio of 1.86. Nodal localization was found in 85%, followed by involvement of the spleen in 20%. The extranodal location is dominated by gastrointestinal involvement (multiple lymphomatous polyposis 25%) and ENT (10%). Almost all patients had ECOG 0-1. Patients had extensive stages (III and IV) of ANN ARBOR. Bone marrow infiltration was found in 35% of cases. The prognostic classification according to MIPI allowed us to classify our cohort into 3 groups as follows: high (n = 12), intermediate (n = 5) and low (n = 3) score. The high proliferation index (+ 50%) in 3 of our patients with the blastoid form in 2 cases were noted. Therapeutically, 19 patients received immuno-chemotherapy according to different protocols: alternating R-CHOP / R-DHAP (7 pts), R-CHOP (4 pts), VR-CAP (4 pts), R-bendamustine (2 pts) and R-mini CHOP (2 pts) with an average number of courses of all protocols 3 (1-6). Maintenance treatment with rituximab every 2 months for 2 years was indicated in 3 patients (2 of whom are still on treatment). The overall response rate is 79%; failure was found in 2 patients. After a median follow-up time of 21 months (5 days-63 months) of the 15 patients in response, 10 are alive in persistent response; late relapse occurred in 2 patients. We deplore 5 deaths including 2 toxic, versus progression of the disease for others. Conclusion: Mantle cell lymphoma is a heterogeneous pathology known to be aggressive and incurable. In spite of recent therapeutic advances, including the provision of immunotherapy (rituximab), the addition of aracytin to high-dose induction and therapeutic intensification followed by autologous-hematopoietic stem cell transplantation and the appearance of new therapies (bortezomib, lénalidomide, ibrutinib ...), the prognosis of the LCM remains poor with a median survival close to 5 years. Citation Format: Fatiha Grifi, Soraya Bougherira, Amel Djenouni. Clinical features and management of mantle cell lymphoma [abstract]. In: Proceedings of the Fourth CRI-CIMT-EATI-AACR International Cancer Immunotherapy Conference: Translating Science into Survival; Sept 30-Oct 3, 2018; New York, NY. Philadelphia (PA): AACR; Cancer Immunol Res 2019;7(2 Suppl):Abstract nr B158.