Mantle cell lymphoma, a single center experience

Introduction: Mantle Cell Lymphoma (MCL) is an indolent B-cell non Hodgkin Lymphoma (NHL) with a worse outcome compared to other low grade NHL. The prognosis of the disease is dictated by various biologic and clinical factors. Although MCL is chemosensitive in newly diagnosed patients, soon it relapses. A significant improvement of its outcome has been achieved by the administration of Rituximab (R) based regimens and the intensification of first line treatment by high dose Cytarabine and autologous stem cell transplantation. Methods: To reveal the experience of our Centre on the diagnosis, treatment and outcome of this difficult to treat lymphoma, we collected data from 51 consecutive patients, diagnosed at our department between 1999 and 2020. Results: Thirty nine (39) men and 12 women with median age 68 years (range 33-88) were diagnosed with MCL. All but one had typical histology. One patient manifested the blastoid variant. Ki67 was available for 39 biopsy: In 57% specimens Ki67 was >30% and in 43% Ki67 was ≤30%. In 84% of cases the performance status at diagnosis was good (PS 0-1) and only 16% showed PS 2-4. The majority of patients (90%) had advanced stage disease (III-IV). High MIPI score was observed in 59%, intermediate in 18% and low in 22%. Elevated lactate dehydrogenase was observed in 58% of cases. Nineteen patients received R - Cyclophosphamide, Hydroxydaunorubicin, Oncovin, Prednisone (R-CHOP), 11 an intensified regimen, 14 patients R -Bendamustin, 5 patients R - Chlorambucil and 2 subjects had no treatment. Response to treatment showed as follow: Complete Response (CR) 52%, Partial Response (PR) 36%, Stable disease (SD) 2% and Progressive Disease (PD) 10%. Among patients who received an intensified treatment, 9 underwent an autologous transplantation. Twenty two patients with responsive disease beneficed of R maintenance. Over a median follow up of 62.8 months (range 0.3 - 262), the median overall survival (OS) was 165 months (range 0.3 - 262). Twenty three out of 44 (52%) patients with responsive disease, relapsed. The median progression free survival (PFS) was 59 months (range 6 - 140). Sixteen out of 23 (69%) patients achieved a 2nd response. The treatments administered were: R-CHOP (6 patients), R-Bendamustin (6 patients), platinum based regimens (3 patients), Ibrutinib (5 patients), corticosteroids (2 patients), R - Chlorambucil (1 patient). The median PFS and OS after the 1st relapse were 26.4 months (range 6.9 - 165) and 27 months (range 0.2 - 233) respectively. Response to 1st line treatment (p 0.0001), lower MIPI score (p 0.001) and intensified regimen (p 0.0004) were correlated with a better survival. Conclusions: Increasing the percentage of 1st complete response by intensifying the 1st line treatment could improve the survival of patients with MCL. Keywords: Indolent non-Hodgkin lymphoma No conflicts of interests pertinent to the abstract.