Introduction: Acute myeloblastic leukaemia (AML) is a haematological malignancy with a poor prognosis, despite significant therapeutic progress. This study presents the results of AML management in Mali according to G...Introduction: Acute myeloblastic leukaemia (AML) is a haematological malignancy with a poor prognosis, despite significant therapeutic progress. This study presents the results of AML management in Mali according to GFAOP recommendations. Methodology: This was a retrospective, cross-sectional study. It included patients aged 0 - 15 years treated in the paediatric oncology unit for AML and followed up between January 2016 and December 2020. Results: During the study period, 85 cases of acute leukaemia were diagnosed in the paediatric oncology unit (including 51 cases of ALL), of which 34 cases of AML were included in this study. The majority were boys (59%). The mean age was 8 years, with extremes of 18 months and 15 years. The mean time to diagnosis was 68 days. In 79% of cases, patients were referred by 1st or 2nd level hospitals. Anaemia was observed in 91% of cases, an infectious syndrome in 68%, haemorrhage in 56% and a tumour syndrome in 85%. The haemogram showed hyperleukocytosis in 15% of cases, thrombocytosis in 22% and severe anaemia in 73%. Death occurred in 85% of cases, most often in the context of sepsis or haemorrhage. Conclusion: AML is probably underestimated in Mali and diagnosis delayed, which may be explained by patient-related factors (lack of knowledge, financial constraints) and a cumbersome referral system. These results suggest the need to implement an appropriate diagnostic and therapeutic strategy, with strong involvement of the political authorities.展开更多
Recurrent acute lymphoblastic leukaemia(ALL) is a common disease for pediatric oncologists and accounts for more deaths from cancer in children than any other malignancy. Although most patients achieve a second remiss...Recurrent acute lymphoblastic leukaemia(ALL) is a common disease for pediatric oncologists and accounts for more deaths from cancer in children than any other malignancy. Although most patients achieve a second remission, about 50% of relapsed ALL patients do not respond to salvage therapy or suffer a second relapse and most children with relapse die. Treatment must be tailored after relapse of ALL, since outcome will be influenced by well-established prognostic features, including the timing and site of disease recurrence, the disease immunophenotype, and early response to retrieval therapy in terms of minimal residual disease(MRD). After reinduction chemotherapy, high risk(HR) patients are clear candidates for allogeneic stem cell transplantation(SCT) while standard risk patients do better with conventional chemotherapy and local therapy. Early MRD response assessment is currently applied to identify those patients within the more heterogeneous intermediate risk group who should undergo SCT as consolidation therapy. Recent evidence suggests distinct biological mechanisms for early vs late relapse and the recognition of the involvement of certain treatment resistance related genes as well cell cycle regulation and B-cell development genes at relapse, all providing the opportunity to search for novel target therapies.展开更多
文摘Introduction: Acute myeloblastic leukaemia (AML) is a haematological malignancy with a poor prognosis, despite significant therapeutic progress. This study presents the results of AML management in Mali according to GFAOP recommendations. Methodology: This was a retrospective, cross-sectional study. It included patients aged 0 - 15 years treated in the paediatric oncology unit for AML and followed up between January 2016 and December 2020. Results: During the study period, 85 cases of acute leukaemia were diagnosed in the paediatric oncology unit (including 51 cases of ALL), of which 34 cases of AML were included in this study. The majority were boys (59%). The mean age was 8 years, with extremes of 18 months and 15 years. The mean time to diagnosis was 68 days. In 79% of cases, patients were referred by 1st or 2nd level hospitals. Anaemia was observed in 91% of cases, an infectious syndrome in 68%, haemorrhage in 56% and a tumour syndrome in 85%. The haemogram showed hyperleukocytosis in 15% of cases, thrombocytosis in 22% and severe anaemia in 73%. Death occurred in 85% of cases, most often in the context of sepsis or haemorrhage. Conclusion: AML is probably underestimated in Mali and diagnosis delayed, which may be explained by patient-related factors (lack of knowledge, financial constraints) and a cumbersome referral system. These results suggest the need to implement an appropriate diagnostic and therapeutic strategy, with strong involvement of the political authorities.
文摘Recurrent acute lymphoblastic leukaemia(ALL) is a common disease for pediatric oncologists and accounts for more deaths from cancer in children than any other malignancy. Although most patients achieve a second remission, about 50% of relapsed ALL patients do not respond to salvage therapy or suffer a second relapse and most children with relapse die. Treatment must be tailored after relapse of ALL, since outcome will be influenced by well-established prognostic features, including the timing and site of disease recurrence, the disease immunophenotype, and early response to retrieval therapy in terms of minimal residual disease(MRD). After reinduction chemotherapy, high risk(HR) patients are clear candidates for allogeneic stem cell transplantation(SCT) while standard risk patients do better with conventional chemotherapy and local therapy. Early MRD response assessment is currently applied to identify those patients within the more heterogeneous intermediate risk group who should undergo SCT as consolidation therapy. Recent evidence suggests distinct biological mechanisms for early vs late relapse and the recognition of the involvement of certain treatment resistance related genes as well cell cycle regulation and B-cell development genes at relapse, all providing the opportunity to search for novel target therapies.
