What are the guideline sources, guidelines and references for Chronic myeloid leukemia ?
Guideline sources The following summarized guidelines for the evaluation and management of chronic myeloid leukemia (CML) are prepared by our editorial team based on guidelines from the British Society for Haematology (BSH 2020), the European Society of Medical Oncology (ESMO 2017), and the American Association of Family Physicians (AAFP 2014). 1 2 3 4 5 5 6 Definition CML is a malignant clonal disorder of hematopoietic stem cells resulting in unregulated growth of myeloid cells in the bone marrow. 4 Epidemiology CML is caused by a chromosomal abnormality termed as Philadelphia chromosome, which results from the presence of BCR-ABL oncogene. 5 Disease course Clinical manifestations include fatigue, weight loss, abdominal fullness, bleeding, purpura, splenomegaly, leukocytosis, anemia, and thrombocytosis. Disease progression may lead to bone https://web.pathway.md/diseases/recFgbW8drJf3mI6K 1/6 6/23/23, 3:26 AM Chronic myeloid leukemia Pathway pain, hepatosplenomegaly, severe anemia, infections, lymphadenopathy, CNS disease, and increased risk of death. 5 Prognosis and risk of recurrence CML is associated with a mortality crude rate of 0.78. 6 Calculator Calculator Eastern Cooperative Oncology G Karnofsky performance status s Guidelines 1. Screening and diagnosis Differential diagnosis: take into consideration the common causes, such as infection, inflammation, and stress response, when evaluating patients with leukocytosis. B 2. Classification and risk stratification Prognostic assessment: consider calculating the EUTOS Long-Term Survival score to inform prognosis. C 3. Diagnostic investigations Diagnostic blood tests: As per ESMO 2017 guidelines, recognize that the diagnosis in most cases can be made on the basis of a characteristic blood count and differential (excessive granulocytosis with a typical left shift of granulopoiesis). E As per AAFP 2014 guidelines, obtain a peripheral blood smear in patients with a WBC count > 20, 000/ L and with associated anemia, thrombocytopenia, thrombocytosis, hepatomegaly, splenomegaly, lymphadenopathy, or constitutional symptoms. B Further evaluation: obtain baseline evaluation with ECG, lipid profile, fasting blood glucose or Hgb A1C, CVD risk assessment, and hepatitis B and C screening in all patients with CML. B 4. Diagnostic procedures Bone marrow aspiration: obtain bone marrow aspiration at diagnosis for full karyotype analysis and to confirm the phase of the disease. B https://web.pathway.md/diseases/recFgbW8drJf3mI6K 2/6 6/23/23, 3:26 AM Chronic myeloid leukemia Pathway Immunohistochemistry: Identify disease progression from chronic phase to blast phase for prognostic and treatment purposes. E Obtain immunocytology by flow cytometry and histochemistry for accurate assessment of immature cells and the distinction between myeloid and lymphoid blast crisis. E Cytogenetic analysis: As per BSH 2020 guidelines, establish the fusion type for molecular monitoring to guide future management. B As per ESMO 2017 guidelines, obtain evaluation of the Philadelphia chromosome, 22q-, or BCR-ABL1 transcripts, or both, in peripheral blood or bone marrow cells to confirm the diagnosis after characteristic blood count and differential results. E Show 3 more Mutational analysis: do not obtain baseline mutational analysis in patients with newly diagnosed CML in chronic phase. D 5. Medical management Management of chronic phase, front-line regimens: As per BSH 2020 guidelines, offer imatinib as first-line therapy in most adult and pediatric patients with CML in chronic phase. A Show 2 more As per ESMO 2017 guidelines, discuss treatment goals with the patient and define them before selecting first-line therapy. A Show 5 more Management of chronic phase, prevention of complications: As per BSH 2020 guidelines, consider interrupting or reducing the dose and titrating slowly to a dose maintaining a major molecular response (< MR3) to manage most side effects of TKIs. B Show 4 more As per ESMO 2017 guidelines, avoid using dasatinib in patients at risk of developing pleural effusions (existing lung disorders or uncontrolled hypertension). Recognize that pulmonary arterial hypertension is a rare complication of dasatinib, and consider offering alternative TKIs in the front-line setting in patients with pre-existing pulmonary arterial hypertension. Recognize that dasatinib also inhibits platelet function, and patients taking concomitant anticoagulants may be at an increased risk of hemorrhagic complications. D Show 3 more Management of chronic phase, treatment monitoring: As per BSH 2020 guidelines, obtain molecular monitoring by reverse transcriptase quantitative PCR and, for typical transcripts, expressed according to the international scale and by molecular response level. A Show 3 more https://web.pathway.md/diseases/recFgbW8drJf3mI6K 3/6 6/23/23, 3:26 AM Chronic myeloid leukemia Pathway As per ESMO 2017 guidelines, recognize that the risk of transformation to accelerated and blast phases is lower in Sokal non-low-risk patients using dasatinib or nilotinib. A Show 4 more Management of chronic phase, treatment discontinuation: As per BSH 2020 guidelines, discuss any case of a patient considering discontinuation at an appropriate hematology multidisciplinary team meeting. B Show 8 more As per ESMO 2017 guidelines, consider discontinuing treatment in individual patients if proper, high-quality, and certified monitoring can be ensured in the presence of the following prerequisites: institutional requirements for safe supervision identification of typical BCR-ABL1 transcripts at diagnosis at least 5 years of TKIs therapy achievement of MR4.5-log reduction stability of deep molecular remission (at least MR4-log reduction) for at least 2 years. C Management of resistant/refractory disease: As per BSH 2020 guidelines, consider switching to an alternative TKI in case of documented treatment failure on first-line therapy. B Show 8 more As per ESMO 2017 guidelines, perform bone marrow examination at the time of treatment failure to allow proper determination of the CML phase and documentation of any clonal evolution. Test CML cells for BCR-ABL1 mutational profile to help guide the selection of the TKIs. E Show 4 more Management of accelerated/blast phase: As per BSH 2020 guidelines: Offer a second-generation TKI in patients with CML in de novo accelerated phase. Consider offering allo-SCT in case of suboptimal response. B Offer allo-SCT in all responding, transplant-eligible patients with CML in blast crisis. B As per ESMO 2017 guidelines: Offer allo-SCT as the only curative option in patients with CML in blast phase. Consider offering allo-SCT early in patients developing accelerated phase during TKI treatment or high- risk patients with insufficient treatment response. B Do not offer allo-SCT in patients with advanced disease with a high transplant risk. Consider offering ongoing drug treatment or best supportive care as options. D Management of infection: obtain evaluation for infection source and initiate empiric broad- spectrum antibiotics in patients with leukemia and fever with neutropenia (< 500/ L). B https://web.pathway.md/diseases/recFgbW8drJf3mI6K 4/6 6/23/23, 3:26 AM Chronic myeloid leukemia Pathway 6. Specific circumstances Pregnant patients: ensure that females with CML wishing to conceive do not have a previous TKI resistance and ideally have maintained MR4 for at least 12 months before considering discontinuation. B Show 3 more 7. Follow-up and surveillance Surveillance for secondary malignancies: obtain serial CBC monitoring and age- and sex- specific cancer screening since leukemia survivors are at increased risk of secondary malignancies. B References 1. A Hochhaus, S Saussele, G Rosti et al. Chronic myeloid leukaemia: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2017 Jul 1;28(suppl_4):iv41-iv51. Open 2. Graeme Smith, Jane Apperley, Dragana Milojkovic et al. A British Society for Haematology Guideline on the diagnosis and management of chronic myeloid leukaemia. Br J Haematol. 2020 Oct;191 2 171 193. Open 3. Amanda S Davis, Anthony J Viera, Monica D Mead. Leukemia: an overview for primary care. 2014 May 1;89 9 731 8.2014 May 1;89 9 731 8. Open 4. Geoffrey C Kabat, Jennifer W Wu, Steven C Moore et al. Lifestyle and dietary factors in relation to risk of chronic myeloid leukemia in the NIH AARP Diet and Health Study. Cancer Epidemiol Biomarkers Prev. 2013 May;22 5 848 54.2013 May;22 5 848 54. Open 5. Rachel Frazer, Alexandra E Irvine, Mary Frances McMullin. Chronic Myeloid Leukaemia in The 21st Century. Ulster Med J. 2007 Jan;76 1 8 17.2007 Jan;76 1 8 17. Open 6. Tumas Beinortas, Ilma Tavoriene, Tadas Zvirblis et al. Chronic myeloid leukemia incidence, survival and accessibility of tyrosine kinase inhibitors: a report from population-based Lithuanian haematological disease registry 2000 2013. BMC Cancer. 2016 Mar 8;16 198.2016 Mar 8;16 198. Open 7. Radich JP, Deininger M, Abboud CN et al. Chronic Myeloid Leukemia, Version 1.2019, NCCN Clinical Practice Guidelines in Oncology. J Natl Compr Canc Netw. 2018 Sep;16 9 1108 1135. Open 8. Geoffrey C. Kabat, Jennifer W. Wu, Steven C. Moore et al. Lifestyle and Dietary Factors in Relation to Risk of Chronic Myeloid Leukemia in the NIH AARP Diet and Health Study. Cancer Epidemiol Biomarkers Prev. 2013 May; 22 5 10.1158/1055 9965.EPI 13 0093. Open 9. Rachel Frazer, Alexandra E Irvine, and Mary Frances McMullin. Chronic Myeloid Leukaemia in The 21st Century. Ulster Med J. 2007 Jan; 76 1 8 17. Open 10. Tumas Beinortas, Ilma Tavorien , Tadas virblis et al. Chronic myeloid leukemia incidence, survival and accessibility of tyrosine kinase inhibitors: a report from population-based Lithuanian haematological disease registry 2000 2013. BMC Cancer. 2016; 16 198. Open https://web.pathway.md/diseases/recFgbW8drJf3mI6K 5/6 6/23/23, 3:26 AM Chronic myeloid leukemia Pathway 11. Michael W Deininger, Neil P Shah, Jessica K Altman et al. Chronic Myeloid Leukemia, Version 2.2021, NCCN Clinical Practice Guidelines in Oncology. J Natl Compr Canc Netw. 2020 Oct 1;18 10 1385 1415. Open 12. Andreas Hochhaus, Delphine R a, Carla Boquimpani et al. Asciminib vs bosutinib in chronic-phase chronic myeloid leukemia previously treated with at least two tyrosine kinase inhibitors: longer-term follow- up of ASCEMBL. Leukemia. 2023 Mar;37 3 617 626. Open https://web.pathway.md/diseases/recFgbW8drJf3mI6K 6/6