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Erythroid response and decrease of WT1 expression after proteasome inhibition by bortezomib in myelodysplastic

Giuliana Alimena1, Massimo Breccia, Pellegrino Musto

  • 1Department of Cellular Biotechnologies and Hematology, Sapienza University of Rome, Via Benevento 6, 00161 Rome, Italy. alimena@bce.uniroma1.it

Leukemia Research
|October 26, 2010
PubMed
Summary

Bortezomib shows modest efficacy in myelodysplastic syndromes (MDS), impacting WT1 gene expression. This proteasome inhibitor warrants further investigation in MDS patients with specific genetic aberrations.

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Area of Science:

  • Hematology
  • Oncology
  • Molecular Biology

Background:

  • Constitutive activation of NF-kB is observed in high-risk myelodysplastic syndromes (MDS) with cytogenetic aberrations.
  • Limited data exist on proteasome inhibitor efficacy in this specific MDS subset.

Purpose of the Study:

  • To evaluate the efficacy and safety of bortezomib as a single agent in patients with myelodysplastic syndromes (MDS).

Main Methods:

  • Nineteen patients with IPSS low/intermediate 1 or intermediate2/high risk MDS received bortezomib (1.3mg/m(2)) on a 1, 4, 8, 11-day schedule every 28 days.
  • Hematologic and non-hematologic toxicities were recorded.
  • Erythroid response was assessed using IWG 2006 criteria.
  • Bone marrow and peripheral blood WT1 gene expression levels were measured.

Main Results:

  • Hematologic toxicity, including grade 3/4 neutropenia and thrombocytopenia, occurred in all patients.
  • Non-hematologic side effects were generally low-grade (1/2 toxicity).
  • Erythroid response was achieved in 21% (4/19) of patients, with 47% (9/19) showing stable disease.
  • Patients achieving erythroid response showed decreased WT1 levels, while stable disease patients exhibited increased WT1 levels.

Conclusions:

  • Bortezomib monotherapy demonstrates modest hematologic efficacy in MDS.
  • Bortezomib appears to influence WT1 gene expression, a marker often elevated in MDS.
  • Further research is needed to explore bortezomib's role in specific MDS patient populations.