Management of CML-blast crisis

Hehlmann, Rüdiger; Saußele, Susanne; Voskanyan, Astghik; Silver, Richard T.

doi:10.1016/j.beha.2016.10.005

Cited by 65 publications

(56 citation statements)

References 93 publications

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“…The 20 participants were aged between 36-75 years (mean 60.9) and had been diagnosed for [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19] years (mean 6.23). 18 had a partner, all had children (four living at home) and 13 were grandparents.…”

Section: Resultsmentioning

confidence: 99%

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Drivers and Barriers to Medication Adherence in Patients with Chronic Myeloid Leukaemia: A Qualitative Study

Mortensen¹,

Mourek

2017

JHOR

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With the introduction of tyrosine kinase inhibitors (TKI), patients with chronic myeloid leukemia (CML) have obtained survival rates close to normal. It may appear paradoxical, then, that medication adherence is suboptimal in some health care settings. As the first of its kind, this study aimed to explore drivers and barriers to TKI treatment adherence in Danish CML patients. A literature study informed the design of qualitative interviews with 20 patients, individually and in focus groups, focusing on their disease perceptions of CML, their health-related quality of life (QoL) and medication adherence. The study showed that many participants had previously switched treatment due to lacking efficacy or intolerance but most felt their current disease burden was tolerable. Anxiety might, however, resurface if treatment stopped working or with the occurrence of infections or side effects, creating a state of 'fragile peace'. To these patients, their role functioning -as professionals, spouses, parents and grandparents -was crucial to uphold a positive self-image and meaningful life. Whether treatment enabled or hindered this was thus decisive to their QoL and medication adherence. Our participants expressed high adherence rates with only one having intentionally non-adhered due to side effects and poor QoL. Most participants felt well-informed about CML and treatment and privileged to receive specialised personal care from the public health care system acting to motivate their medication adherence. As a novel finding, this study indicates that the prospect of treatment-free remission may positively affect adherence. We suggest this should be explored in future studies.

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Section: Resultsmentioning

confidence: 99%

“…If untreated, CP-CML will progress to an accelerated phase (AP-CML) and a fatal blast phase (BP-CML) in three to five years [1,4,5].…”

Section: Introductionmentioning

confidence: 99%

Drivers and Barriers to Medication Adherence in Patients with Chronic Myeloid Leukaemia: A Qualitative Study

Mortensen¹,

Mourek

2017

JHOR

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“…However, a significant minority of patients will have their disease transform to BP; this occurs less frequently in patients treated with second‐generation TKIs as their initial therapy . The median survival in patients with CML‐BP is < 12 months . The pathobiology of CML‐BP involves multiple intermingling pathways with genetic and epigenetic aberrations involved in transformation, but to our knowledge is not fully understood …”

Section: Introductionmentioning

confidence: 99%

“…1 The median survival in patients with CML-BP is < 12 months. [2][3][4][5] The pathobiology of CML-BP involves multiple intermingling pathways with genetic and epigenetic aberrations involved in transformation, but to our knowledge is not fully understood. 6 Although the poor prognosis of patients with CML-BP is well recognized, to our knowledge the predictive prognostic features in the era of TKIs have been addressed only briefly.…”

Section: Introductionmentioning

confidence: 99%

Prognostic factors and survival outcomes in patients with chronic myeloid leukemia in blast phase in the tyrosine kinase inhibitor era: Cohort study of 477 patients

Jain

Kantarjian

Ghorab

et al. 2017

Cancer

126

127

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Background Outcomes in blast phase CML (CML-BP) are historically dismal. We sought to analyse the characteristics, prognostic factors and survival outcomes in patients with CML-BP in the TKI era. Methods All patients with CML-BP (n=477) were treated with a TKI at some point during the course of their CML. Cox proportional hazard models identified characteristics which predicted for survival. Overall survival (OS) and failure free survival (FFS) were assessed. Optimal cut off points for specific parameters, were identified using CART (classification and regression tree). Results Median age was 53 years (range, 16 to 84); 64% were male. Eighty percent were initially diagnosed in chronic phase CML (CML-CP) median 41 months (0.7 to 298 months) before transformation to CML-BP. De-novo CML-BP occurred in 71 patients. Seventy two percent patients received TKI therapy prior to CML-BP. Initial therapy for CML-BP included, TKI alone (35%), TKI with chemotherapy (46%) and non-TKI therapies (19%). The median OS was 12 months and median FFS was 5 months. In multivariate analysis, myeloid immunophenotype, prior TKI, age ≥58 years, LDH ≥1227 IU/L, platelet count <102 K/μL, no stem cell transplantation (SCT), blast phase from CP/AP and presence of chromosome 15 aberrations predicted for a significantly increased risk of death. Achievement of major hematologic response and/or complete cytogenetic response to first line treatment predicted for better survival. Combination of TKI with intensive chemotherapy followed by stem cell transplant confer the best outcome. Conclusions Patients with CML-BP continue to pose a therapeutic challenge, have dismal outcomes and require newer treatment approaches.

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“…FISH analysis of cells sorted into lineage‐specific populations and genomic analysis are feasible and can be included in routine diagnostic procedures and will undoubtedly lead to better characterization of these BCR‐ABL1‐positive leukemic subtypes. Although the optimal therapy for subtypes with multilineage involvement is yet to be defined, we believe the current standard practice is HCST analogous to CML that presents in BP …”

Section: Discussionmentioning

confidence: 99%

Evidence for BCR/ABL1‐positive T‐cell acute lymphoblastic leukemia arising in an early lymphoid progenitor cell

Ragg

Zehentner²,

Loken³

et al. 2019

Pediatric Blood & Cancer

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BCR‐ABL1‐positive leukemias have historically been classified as either chronic myelogenous leukemia or Ph+ acute lymphoblastic leukemia. Recent analyses suggest there may be a wider range of subtypes. We report a patient with BCR‐ABL1 fusion positive T‐cell ALL with a previously undescribed cell distribution of the fusion gene. The examination of sorted cells by fluorescence in situ hybridization showed the BCR‐ABL1 fusion in the malignant T cells and a subpopulation of the nonmalignant B cells, but not nonmalignant T cells or myeloid or CD34+ progenitor cells providing evidence that the fusion may have occurred in an early lymphoid progenitor.

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Management of CML-blast crisis

Cited by 65 publications

References 93 publications

Drivers and Barriers to Medication Adherence in Patients with Chronic Myeloid Leukaemia: A Qualitative Study

Drivers and Barriers to Medication Adherence in Patients with Chronic Myeloid Leukaemia: A Qualitative Study

Prognostic factors and survival outcomes in patients with chronic myeloid leukemia in blast phase in the tyrosine kinase inhibitor era: Cohort study of 477 patients

Evidence for BCR/ABL1‐positive T‐cell acute lymphoblastic leukemia arising in an early lymphoid progenitor cell

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