Chronic Myelogenous Leukemia, Version 1.2015

Abstract

OverviewChronic myelogenous leukemia (CML) accounts for 15% of adult leukemias. The median age at disease onset is 67 years; however, SEER statistics show that CML occurs in all age groups. 1 In 2014, an estimated 5980 people will be diagnosed with CML in the United States, and 810 people will die from the disease. CML is characterized by the presence of Philadelphia chromosome (Ph) resulting from a reciprocal translocation between chromosomes 9 and 22 [t(9;22)]. This translocation, t(9;22), results in the head-to-tail fusion of the breakpoint cluster region (BCR) gene on chromosome 22 at band q11 and the Abelson murine leukemia (ABL1) gene located on chromosome 9 at band AbstractChronic myelogenous leukemia (CML) is usually diagnosed in the chronic phase. Untreated chronic phase CML will eventually progress to advanced phase (accelerated or blast phase) CML. Tyrosine kinase inhibitors (TKIs) have been shown to induce favorable response rates in patients with accelerated and blast phase CML. The addition of TKIs to chemotherapy has also been associated with improved outcomes in patients with blast phase CML. Allogeneic hematopoietic stem cell transplant remains a potentially curative option for patients with advanced phase CML, although treatment with a course of TKIs will be beneficial as a bridge to transplant. This manuscript discusses the recommendations outlined in the NCCN Guidelines for the diagnosis and management of patients with advanced phase CML. (J Natl Compr Canc Netw 2014;12:1590-1610 NCCN Categories of Evidence and ConsensusCategory 1: Based upon high-level evidence, there is uniform NCCN consensus that the intervention is appropriate. Category 2A: Based upon lower-level evidence, there is uniform NCCN consensus that the intervention is appropriate. Category 2B: Based upon lower-level evidence, there is NCCN consensus that the intervention is appropriate. Category 3: Based upon any level of evidence, there is major NCCN disagreement that the intervention is appropriate. © National Comprehensive Cancer Network, Inc. 2014, All rights reserved. The NCCN Guidelines and the illustrations herein may not be reproduced in any form without the express written permission of NCCN. Disclosures for the NCCN Chronic Myelogenous Leukemia PanelAt the beginning of each NCCN Guidelines panel meeting, panel members review all potential conflicts of interest. NCCN, in keeping with its commitment to public transparency, publishes these disclosures for panel members, staff, and NCCN itself.Individual disclosures for the NCCN Chronic Myelogenous Leukemia Panel members can be found on page 1610. (The most recent version of these guidelines and accompanying disclosures are available on the NCCN Web site at NCCN.org.)These guidelines are also available on the Internet. For the latest update, visit NCCN.org. q34. 3 The product of the BCR-ABL1 fusion gene, p210, which is a fusion protein with deregulated tyrosine kinase activity, plays a central role in the pathogenesis of CML. Another fusion protein, p190, is also produc...

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