18. Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia: Current Treatment Status and Perspectives

  1. Stefan Faderl MD Associate Professor and
  2. Hagop Kantarjian MD Chairman Professor
  1. Olga Sala-Torra MD and
  2. Jerald P. Radich MD

Published Online: 4 JAN 2011

DOI: 10.1002/9781444327359.ch18

Leukemias: Principles and Practice of Therapy

Leukemias: Principles and Practice of Therapy

How to Cite

Sala-Torra, O. and Radich, J. P. (2010) Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia: Current Treatment Status and Perspectives, in Leukemias: Principles and Practice of Therapy (eds S. Faderl and H. Kantarjian), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781444327359.ch18

Editor Information

  1. Department of Leukemia, University of Texas, MD Anderson Cancer Center, Houston, TX, USA

Author Information

  1. Clinical Research Division, Fred Hutchinson Cancer Research Center, Seattle, Washington, USA

Publication History

  1. Published Online: 4 JAN 2011
  2. Published Print: 26 NOV 2010

ISBN Information

Print ISBN: 9781405182355

Online ISBN: 9781444327359

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Keywords:

  • Philadelphia chromosome;
  • Bcr-Abl;
  • tyrosine kinase;
  • tyrosine kinase inhibitors;
  • hematopoietic stem cell transplant

Summary

Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph+ ALL) accounts for approximately 25–30% of acute lymphoblastic leukemia (ALL) diagnosed in adults, and < 5% of ALL diagnosed in pediatric patients. The Philadelphia chromosome is a well-characterized recurrent chromosomal abnormality that confers adverse prognosis to ALL. Although cases of Ph+ ALL achieve complete remission with conventional chemotherapy regimes in 70–90% of patients, long-term survival does not surpass 20%, reflecting a high rate of relapse and failure of salvage regimens. Thus, patients diagnosed with Ph+ ALL are considered hematopoietic stem cell transplant (HCT) candidates if a suitable donor is available. The initial treatment of Ph+ ALL has been dramatically changed by the introduction of Abl tyrosine kinase inhibitors (TKI). Used as single agents, or in combination with chemotherapy, complete remissions are common, although, even with combination approaches, relapse is the rule. A more complete understanding of the biology of the Ph chromosome is likely needed in order to cure patients who cannot receive a HCT.