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EHA 2018 | Genetic mutations identify patients with poor prognosis in splenic MZL

Jul 6, 2018

On Friday 15 thJune an oral abstract session took place at the 23 rdCongress of the European Hematology Association(EHA). Abstract S103was presented by Francesca Guidetti, Institute of Oncology Research, Bellinzona, Switzerland, provided an interim analysis of the IELSG46 study. This study aimed to investigate the impact of different biological aspects of splenic marginal zone lymphoma (SMZL) on disease survival.

Dr Guidetti explained that there are 133 genes that are recurrently mutated in SMZL and lymphomas. Molecular genetics have improved the knowledge of MZL pathogenesis and although the disease is indolent, the disease is still incurable. It has been found that molecular features of SMZL associated with prognosis are; immunoglobulin gene mutations, 7q deletion, NOTCH2mutations, KLF2mutation and TP53disruption.

Study Overview

  • More than 30 centers provided spleen tissues of patients diagnosed with SMZL before Dec 31 st2010
  • The relative survival of the study cohort after a median follow-up of 9.2 years was 85% and the median overall survival (OS) was 17.1 years
  • The 10-year relative survival for NOTCH2and KLF2(n = 44) was 68.8%, P< 0.001
  • The 10-year relative survival for SPENand KMT2D(n = 35) was 86.5%, P= 0.311
  • The 10-year relative survival for others (n = 167) was 82.3%, P= 0.092

Dr Guidetti concluded that patients with the TP53 mutation (10%) had a lower probability of OS. Patients with NOTCH2/KLF2mutations have a lower relative survival compared to the matched general population.

  1. Guidetti F. TP53 Mutations, but not NOTCH2 or KLF2 mutations, identify patients with poor prognosis in splenic marginal zone lymphoma – interim analysis of the IELSG46 study. Abstract S103. 23 rdCongress of EHA, Stockholm, Sweden