Puppet masters of B-cell progenitor acute lymphoblastic leukemia: The preB cell receptor and the interleukin 7 receptor α

Eur J Immunol. 2023 Apr;53(4):e2250093. doi: 10.1002/eji.202250093. Epub 2023 Mar 9.


B-cell progenitor acute lymphoblastic leukemia (BCP-ALL) is enriched for a preB cell phenotype, hinting at a specific vulnerability of this cell stage. Two signaling pathways via the preB cell receptor (preBCR) and the interleukin 7 receptor α (IL-7Rα) chain govern the balance between differentiation and proliferation at this stage and both receptor pathways are routinely altered in human BCP-ALL. Here, we review the immunobiology of both the preBCR as well as the IL-7Rα and analyze the human BCP-ALL spectrum in the light of these signaling complexes. Finally, we present a terminology for preBCR signaling modules that distinguishes a pro-proliferative "phase-I" module from a pro-differentiative "phase-II" module. This terminology might serve as a framework to better address shared oncogenic mechanics of preB cell stage BCP-ALL.

Keywords: BCP-ALL; IL-7Rα; leukemogenesis; oncogenesis; preBCR.

Publication types

  • Review
  • Research Support, Non-U.S. Gov't

MeSH terms

  • B-Lymphocytes / metabolism
  • Burkitt Lymphoma*
  • Humans
  • Interleukin-7 / metabolism
  • Pre-B Cell Receptors / genetics
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma*
  • Receptors, Interleukin-7 / metabolism


  • Pre-B Cell Receptors
  • Receptors, Interleukin-7
  • Interleukin-7