scholarly journals Additional cytogenetic abnormalities and variant t(9;22) at the diagnosis of childhood chronic myeloid leukemia: The experience of the International Registry for Chronic Myeloid Leukemia in Children and Adolescents

Cancer ◽  
2017 ◽  
Vol 123 (18) ◽  
pp. 3609-3616 ◽  
Author(s):  
Frédéric Millot ◽  
Christelle Dupraz ◽  
Joelle Guilhot ◽  
Meinolf Suttorp ◽  
Françoise Brizard ◽  
...  
Author(s):  
meryeme abddaoui ◽  
ahmed faleh ◽  
imane tlemçani ◽  
sara ben miloud ◽  
moncef amrani hassani

Pediatric chronic myeloid leukemia is a rare entity (2-5% of childhood leukemias) classified as a myeloproliferative neoplasia characterized by the presence of the BCR-ABL fusion gene, the oncogenic translocation product (9; 22) responsible for the disease through its deregulated tyrosine kinase activity.


Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 1576-1576
Author(s):  
Frédéric Millot ◽  
Joelle Guilhot ◽  
Meinolf Suttorp ◽  
Anne Sophie Meunier ◽  
Gunes Adalet Meral ◽  
...  

Abstract Aims: To determine in children and adolescents with chronic myeloid leukemia (CML) in chronic phase (CP) treated with imatinib front line, (i) the probability of switch to a second line therapy, (ii) to characterize the reasons and the type of switch and (iii) to determine the impact of the switch on outcome. Methods: Children and adolescents (<18 years) with CML diagnosed later than the year 2000 and enrolled in the international registry for childhood CML (I-CML-Ped Study) were the subjects of the study. Results: The I-CML-Ped study enrolled 301 children and adolescents with CML in CP treated with imatinib front line. Among them 112 patients subsequently switched to a second line therapy (median duration of imatinib treatment before the switch: 16 months [range: 1-111]).The probability of switch at 38 months was 50% (95% CI: 29-60). Primary resistance was the cause of switch in 47% of the patients: failure to achieve complete hematologic response (CHR, 1%), complete cytogenetic response (CCR, 20%) or major molecular response (MMR, 24%); not detailed (2%). A loss of response (CHR loss [2%] or CCR loss [4%] or MMR loss [13%]) or progression were the cause of switch in 19% and 4% of the patients, respectively. Occurrence of non hematologic toxicity (mainly muscle-skeleton pain) was the cause of switch in 10% of the patients. The reason of switch was the physician's choice in 20% of the patients (switch to hematopoietic stem cell transplantation [HSCT] while the patients were in MMR or deeper molecular response). The second line therapy consisted of second generation tyrosine kinase inhibitors (63%), chemotherapy (4%) or HSCT (33%). With a median follow up of 38 months (range: 2-150), overall, 8 deaths were recorded among switching patients: all were patients transplanted for acute phase (4 patients), hematologic resistance (1 patient), loss of hematologic response (1 patient) or physician's choice (2 patients). The causes of death were treatment related mortality (7 patients) and relapse (1 patient). One death only was recorded among the non switching patients. The probability of overall survival at 48 months was 90% (95% CI: 81% - 95%) for switching patients and 98% (95% CI: 89% - 100%) (p=0.005) for the non switching patients. Conclusion: Treatment failure is the main reason for a switch to a second line therapy in children and adolescents treated with imatinib front line. Efficacy of second line therapy still needs improvement. Disclosures No relevant conflicts of interest to declare.


2012 ◽  
Vol 53 (12) ◽  
pp. 2430-2433 ◽  
Author(s):  
K. C. Lakshmaiah ◽  
Rohan Bhise ◽  
Samit Purohit ◽  
Linu J. Abraham ◽  
D. Lokanatha ◽  
...  

Blood ◽  
2019 ◽  
Vol 133 (22) ◽  
pp. 2374-2384 ◽  
Author(s):  
Nobuko Hijiya ◽  
Meinolf Suttorp

AbstractEvidence-based recommendations have been established for treatment of chronic myeloid leukemia (CML) in adults treated with tyrosine kinase inhibitors (TKIs), but the rarity of this leukemia in children and adolescents makes it challenging to develop similar recommendations in pediatrics. In addition to imatinib, which was approved for pediatric CML in 2003, the second-generation TKIs dasatinib and nilotinib were recently approved for use in children, expanding the therapeutic options and pushing allogeneic stem cell transplantation to a third-line treatment of most pediatric cases. Yet, without sufficient data on efficacy and safety specific to pediatric patients, the selection of a TKI continues to rely on clinical experience in adults. Here, we present 4 case scenarios highlighting common yet challenging issues encountered in the treatment of pediatric CML (suboptimal response, poor treatment adherence, growth retardation, and presentation in advanced phases). Limited experience with very young children, the transition of teenagers to adult medicine, and the goal of achieving treatment-free remission for this rare leukemia are additional significant obstacles that require further clinical investigation through international collaboration.


2019 ◽  
Vol 66 (9) ◽  
Author(s):  
Uma Athale ◽  
Nobuko Hijiya ◽  
Briana C. Patterson ◽  
John Bergsagel ◽  
Jeffrey R. Andolina ◽  
...  

Sign in / Sign up

Export Citation Format

Share Document