A British Society for Haematology Guideline on the diagnosis and management of chronic myeloid leukaemia

Abstract

The management of chronic myeloid leukaemia (CML) has seen considerable change in the last several years. The objective of this guideline is to provide healthcare professionals with clear guidance on the investigation and management of CML in adults and children. Methodology This guideline was compiled according to the British Society for Haematology (BSH) process described at http://www.b-sh.org.uk/guidelines. The Grading of Recommendations Assessment (GRADE) nomenclature was used to evaluate levels of evidence and to assess the strength of recommendations. The GRADE criteria can be found at http:// www.gradeworkinggroup.org.

Interrogating the molecular genetics of chronic myeloproliferative malignancies for personalized management in 2021

“…RNA-seq: RNA-sequencing; NGS: next-generation sequencing; MRD: measurable residual disease; ITH: intratumoral heterogeneity; PCR: polymerase chain reaction. (Adapted with permission from Radich JP et al 7 ) the intron between exons e13 and e14, or in the intron between exons e14 and e15. 6 A break in the former intron yields an e13a2 mRNA junction and a break in the latter intron yields an e14a2 junction.…”

“…RNA-seq: RNA-sequencing; NGS: nextgeneration sequencing; MRD: measurable residual disease; ITH: intratumoral heterogeneity; PCR: polymerase chain reaction. (Adapted with permission from Radich JP et al 7 )…”

“…[4][5][6] The greatest advance has been in CML, with a significant proportion of patients being able to achieve a major molecular response (MMR or MR3; BCR-ABL1 ≤0.1% on the International Scale) following treatment with an ABL1-tyrosine kinase inhibitor (TKI), leading to these patients having lifespans indistinguishable from those of the general population, although the time by which this response milestone should be reached remains controversial. 7 Qualified success with significant symptomatic benefit and modest gains in survival have also been achieved in people with MF and SM, following treatment with JAK inhibitors and KIT inhibitors, respectively. 6 Some of these achievements have been facilitated by the rational integration of next-generation sequencing (NGS) assays, high-sensitive polymerase chain reaction (PCR) assays on DNA or RNA (sensitivity 0.01%-0.1%), and single-cell analyses, in efforts to improve personalized treatment approaches.…”

The chronic myeloid leukaemia story in the United Kingdom since 1960

“…Known as TASTER and led by Mhairi Copland, the design is similar to the LI‐1 strategy for acute myeloid leukaemia (AML), and will initially test tazemetostat, targeting EZH2, in patients resistant to at least two TKIs and ineligible for transplantation. Graeme Smith (formerly Leeds), Jane Apperley, Mhairi Copland and colleagues have recently published a BSCH guideline for current CML management orientated to UK practice, 49 which builds on and extends many of the recent European LeukemiaNet recommendations 50 …”

Another set of guidelines for chronic myeloid leukaemia

“…There are also other lesser differences between these two sets of recommendations. Why therefore is another set of guidelines, the subject of this commentary, needed from a panel of 15 leading UK CML experts? 4 The UK may differ from other countries in that CML care is spread across ~140 National Health Service hospitals (some of which see less than five new cases per annum) where treatment is free‐at‐the‐point‐of‐use; other factors may include the regulatory role of NICE, the generally excellent rapport with primary care settings and the care for younger patients entirely by paediatric haemato‐oncologists. Reliable molecular monitoring of BCR‐ABL1 is available nationwide from ~22 UK NHS laboratories all reporting to an IS baseline and participating in a well‐run quality assurance scheme, integrated with that of European colleagues.…”