Chronic-Phase Chronic Myelogenous Leukemia
Note: Some citations in the text of this section are followed by a level of evidence. The PDQ editorial boards use a formal ranking system to help the reader judge the strength of evidence linked to the reported results of a therapeutic strategy. (Refer to the PDQ summary on Levels of Evidence for more information.)
Treatment Options for Chronic-Phase Chronic Myelogenous Leukemia
High-Stage Childhood Lymphoblastic Lymphoma
Patients with high-stage (stage III or IV) lymphoblastic lymphoma have long-term survival rates higher than 80%.[1] Unlike other pediatric non-Hodgkin lymphoma (NHL), it has been shown that lymphoblastic lymphoma responds much better to leukemia therapy with 2 years of therapy than with shorter, intensive, pulsed chemotherapy regimens.[1,2,3] Involvement of the bone marrow may lead to confusion as to whether the patient has lymphoma or leukemia. Traditionally, patients with more than 25% marrow...
Read the High-Stage Childhood Lymphoblastic Lymphoma article > >
- Targeted therapy with tyrosine kinase inhibitors.
- High-dose therapy followed by allogeneic bone marrow transplant (BMT) or stem cell transplantation (SCT).
- Biologic therapy with or without chemotherapy.
- Hydroxyurea.
- Splenectomy may be required and useful in patients having hematologic problems and physical discomfort from a massive spleen.
Targeted therapy with tyrosine kinase inhibitors
A trial randomizing 1,106 previously untreated patients to imatinib mesylate or to interferon plus cytarabine documented a 76% complete cytogenetic response rate with imatinib mesylate versus 14% for interferon plus cytarabine at a median follow-up of 19 months.[1,2][Level of evidence: 1iiDiii] At 18 months, 96.7% of the imatinib group had avoided progression to accelerated-phase or blast crisis compared with 91.5% of the interferon plus cytarabine group (P < .001). Because 90% of the combination group had switched to imatinib by 18 months (mostly because of intolerance of side effects), a survival difference may never be observed. By the 5-year median follow-up of this trial, imatinib mesylate induced complete cytogenetic response in more than 80% of the participants, with the annual rate of progression to accelerated phase or blast crisis dropping from 2% in the first year to less than 1% in the fourth year.[2] In addition, the overall survival (OS) rate for all patients at 5 years is 89%, with fewer than 50% of all deaths (4.5%) caused by CML. More than 90% of completely responding patients still show detectable evidence of the BCR/ABL translocation, usually by RT-PCR or by fluorescence in situ hybridization of progenitor cell cultures.[3,4,5] The clinical implication of this finding after 10 years or more is unknown, but these results have changed clinical practice.
Tyrosine kinase inhibitors with greater potency and selectivity for BCR/ABL than imatinib have been evaluated in newly diagnosed patients with CML. In a randomized prospective study of 846 patients comparing nilotinib with imatinib, the rate of major molecular response at 12 months was 43% and 44% for 2-dose schedules of nilotinib and 22% for imatinib (P < .001 for both comparisons). The rate of complete cytogenetic response at 12 months was 80% and 78% for 2-dose schedules of nilotinib and 65% for imatinib (P < .001 for both comparisons).[6][Level of evidence: 1iiDiv] Progression to accelerated phase or blast crisis occurred in 11 patients on imatinib (4%) but only occurred in 2 patients (<1%, P = .01) and 1 patient (<1%, P = .004) for the patients on 2-dose schedules of nilotinib.[6]
WebMD Public Information from the National Cancer Institute
Today in Cancer
WebMD Special Sections
Health Solutions From Our Sponsors
©2005-2012 WebMD, LLC. All rights reserved.
WebMD does not provide medical advice, diagnosis or treatment. See additional information.