Combined analysis of IKZF1 deletions and CRLF2 expression on prognostic impact in pediatric B-cell precursor acute lymphoblastic leukemia

Leuk Lymphoma. 2021 Feb;62(2):410-418. doi: 10.1080/10428194.2020.1832668. Epub 2020 Oct 15.

Abstract

This study aimed to investigate the combined impact of IKZF1 deletions/high expression of CRLF2 on the prognosis of pediatric B-cell precursor acute lymphoblastic leukemia (BCP-ALL). IKZF1 deletions and CRLF2 expression were assessed in bone marrow samples from 117 children with newly diagnosed BCP-ALL. Sixteen (13.7%) patients were found to harbor IKZF1 deletions, which was associated with inferior outcomes. The event-free survival (EFS) for patients with high -CRLF2 expression was significantly worse than that for low -CRLF2 expression. Moreover, combined modeling of IKZF1+ /CRLF2high identified 7.8% of cases as the highest risk subgroup (7-year EFS 33.3 ± 15.7%). In a multivariate analysis, IKZF1+ /CRLF2high remained a strong independent prognostic factor for EFS (HR: 14.263, p = 0.019). IKZF1 deletions and high -CRLF2 expression were associated with inferior outcomes, and the coexistence of IKZF1+ /CRLF2high had a significant impact on an integrated prognostic model for high-risk BCP-ALL.

Keywords: Acute lymphoblastic leukemia; CRLF2; IKZF1; prognosis.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • B-Lymphocytes
  • Burkitt Lymphoma*
  • Child
  • Humans
  • Ikaros Transcription Factor / genetics
  • Precursor B-Cell Lymphoblastic Leukemia-Lymphoma* / diagnosis
  • Precursor B-Cell Lymphoblastic Leukemia-Lymphoma* / genetics
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma*
  • Prognosis
  • Receptors, Cytokine / genetics

Substances

  • CRLF2 protein, human
  • IKZF1 protein, human
  • Receptors, Cytokine
  • Ikaros Transcription Factor