Abstract Topic: 19. Aggressive Non-Hodgkin lymphoma - Clinical
Background: Primary CNS lymphoma (PCNSL) is a highly aggressive non-Hodgkin lymphoma that originates in the brain, cerebrospinal fluid, spinal cord, or eyes. Because the affected areas of the CNS differ from patient to patient, clinical signs and symptoms of the disease are variable, and diagnosis requires a high level of suspicion.
Aims: We aim to report our data on the use of the R-MPV protocole (Rituximab, Methotrexate, Procarbazine, Vincristine) followed by a reduced dose whole-brain radiotherapy in the treatment of PCNSL in real word settings.
Methods: This is a descriptive retrospective study conducted on 10 immunocompetent patients, at the clinical hematology department of the Ibn Sina teaching hospital in Rabat over 6 years (January 2017-September 2022).
Results: In the cohort studied, the median age was 50 years [19 years – 78 years], with a M/F sex ratio of 0.6 [6 females – 4 males].The clinical examination found a deteriorated general condition in the majority of patients (50% of the patients had a PS score of 4, 20% had a PS score of 3). Four patients had a pyramidal syndrome, three a cerebellar syndrome, two had phasic disorders, one had a Parinaud’s syndrome and one had intracranial hypertension. Sus and sub-tentorial locations were the most common in imaging (30%).
The anatomopathological study showed a diffuse large B cell lymphoma (DLBCL) non germinal centre type in 9 patients, and an NOS T cell lymphoma in one patient.
The therapeutic protocol consisted in the administration of Rituximab for B cell lymphomas, associated to Methotrexate, Procarbazine and Vincristine (R-MPV), and MPV for T cell lymphoma. A reduced dose whole-brain radiotherapy (rdWBRT) consolidation and high dose Cytarabine was performed. Complete remission was achieved in 6 patients (2 after 5 R-MPV cycles and 4 after 7 cycles).
The median follow-up was 15.7 months, and the overall survival (OS) was 50%. The overall response to treatment (OR) was 70%. Progression free survival (PFS) at 6 months was 60%, and at 12 months 50% (1 primary refractory patient, 1 relapse and 1 progression).
The complications were mainly hematological toxicities, with 80% grade III-IV neutropenia and thrombopenia.
Summary/Conclusion: Following our experience, an advanced age at diagnosis, low PS score and late diagnosis are poor prognosis factors. The R-MPV protocol associated to consolidation rdWBRT and Cytarabine was associated with high response rate and minimal neurotoxicity, even in elderly patients.
Keywords: Radiotherapy, Non-Hodgkin’s lymphoma, CNS lymphoma