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

On Friday 15th June an oral abstract session took place at the 23rd Congress of the European Hematology Association (EHA). Abstract S103 was 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, NOTCH2 mutations, KLF2 mutation and TP53 disruption.

Study Overview
  • More than 30 centers provided spleen tissues of patients diagnosed with SMZL before Dec 31st 2010
  • 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 NOTCH2 and KLF2 (n = 44) was 68.8%, P < 0.001
  • The 10-year relative survival for SPEN and 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/KLF2 mutations have a lower relative survival compared to the matched general population.


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. 23rd Congress of EHA, Stockholm, Sweden


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