Kloke, O.

Simonsson, B.

Stahel, R. A.

RERO DOC Digital Library

ESMO Minimum Clinical Recommendations for the diagnosis,treatment and follow-up of chronic myelogenous leukemia(CML)Incidence. The incidence according to the SEER data is 1.38/100 000per year. The mortality is 0.74/100 000 per year. Themedian age at diagnosis is around 60 years.Diagnosis. Myeloid hyperplasia associated with splenomegaly and neu-trophil leukocytosis, thrombocytosis and basophilia are typi-cal features of CML in its initial chronic phase.. In almost all patients, blood and marrow cells carry thespecific chromosomal translocation t(9;22), the Philadelphiachromosome, or its variants.Staging and risk assessment. The major risk is the transformation of the initial chronicinto the blastic phase of the disease characterized by >_30%blasts in blood or marrow, or >_50% blasts and promyelo-cytes in marrow, or extra-medullar blastic infiltration. Anaccelerated phase (transitional from chronic to blasticphase) is defined as 15–29% blasts in marrow or blood, or30–49% blasts plus promyelocytes in marrow.. Prognostic scores based on age, spleen size, and blood cellcounts have been developed that allow the discrimination ofpatient subgroups with different prognosis on cytoreductivechemotherapy and on interferon, respectively.Treatment. The only curative treatment available is allogeneic stem celltransplantation. Younger patients, especially those withunfavorable risk factors, should be considered for this treat-ment at diagnosis. The patient should be involved in thedecision process, which must balance the chance of cureagainst the risk of transplant-related mortality.. The tyrosine kinase inhibitor imatinib is highly effective ininducing hematologic and cytogenetic remissions [I, A].Imatinib (400mg p.o. daily) is first-line standard therapybecause of better tolerance, a much higher cytogeneticresponse rate, and a lower progression rate to accelerated orblastic phase compared with interferon-/ara-C-based therapy.However, its long term efficacy is not yet known [I, A].. In good prognosis patients, interferon-a at doses of3–5MU/m2 daily may be considered as an alternativetreatment.. The clinical benefit of intensive chemotherapy with or with-out autologous stem cell transplantation is not proven.. If the treatment goal is only to relieve symptoms, hydro-xyurea may be considered [II, A].Follow-up. Patients are followed by blood cell counts once weeklyduring the first weeks of therapy and every 1–2 monthslater on. Bone marrow cytogenetics (and/or quantitativePCR of bcr/abl) should be performed in imatinib-treated orinterferon-treated patients every 6 months. Patients relapsingafter allotransplant may still have curative options, e.g.donor lymphocyte transfusions, and require more frequentcontrols.NoteLevels of Evidence [I–V] and Grades of Recommendation[A–D] as used by the American Society of Clinical Oncologyare given in square brackets. Statements without grading wereconsidered justified standard clinical practice by the expertsand the ESMO faculty.Literature1. Sokal JE, Cox EB, Baccarani M et al. Prognostic discrimination in“good risk” chronic granulocytic leukaemia. Blood 1984; 63:789–799.2. Hasford J, Pfirrman M, Hehlmann R et al. A new prognosticscore for survival of patients with chronic myeloid leukaemiatreated with interferon alfa. J Natl Cancer Inst 1998; 90:850–858.3. Silver RT, Woolf SH, Hehlmann R et al. An evidence-based analysisof the effect of Busulfan, Hydroxyurea, Interferon and allogeneicbone marrow transplantation in treating the chronic phase of chronicmyeloid leukaemia: Developed for the American Society of Hematol-ogy. Blood 1999; 94: 1517–1536.4. Enright H, McGlave P. Chronic myelogenous leukaemia. In HoffmanR, Benz EJ, Shattil SJ (eds): Hematology: Basic Principles and Prac-tice. Philadelphia: Churchill Livingstone 2000.5. Kolb HJ, Mittermuller J, Clemm C et al. Donor leukocyte transfusionsfor treatment of recurrent chronic myelogenous leukaemia in marrowtransplant patients. Blood 1990; 76: 2462–2465.6. O’Brien SG, Guilhot F, Larson RA et al. Imatinib compared withinterferon and low-dose cytarabine for newly diagnosed chronic-phasechronic myeloid leukemia. N Engl J Med 2003; 348: 994–1004.Annals of Oncology 16 (Supplement 1): i52–i53, 2005doi:10.1093/annonc/mdi807q 2005 European Society for Medical OncologyCoordinating authors for the ESMO Guidelines Task Force: B. Simons-son1, O. Kloke2 & R. A. Stahel31Invited author, Dept. Hematology, University Hospital, Uppsala, Sweden;2Assigned task force member, Elisabeth Krankenhaus GmbH, Abt.Onkologie/Ha¨matologie, Ro¨ntgenstrasse 10, 45 661 Recklinghausen,Germany; 3Assigned task force member, Div. of Oncology, UniversityHospital, Ra¨mistr. 100, CH-8091 Zu¨rich, SwitzerlandApproved by the ESMO Guidelines Task Force: August 2003, last updateDecember 2004.Correspondence to:ESMO Guidelines Task ForceESMO Head OfficeVia La Santa 7CH-6962 LuganoSwitzerlandi53

ESMO Minimum Clinical Recommendations for the diagnosis, treatment and follow-up of chronic myelogenous leukemia (CML)

Abstract

Similar works

Full text

Available Versions

RERO DOC Digital Library