Childhood leukemia bottleneck phenomenon is the most mysterious corollary of the prenatal origin discovery of leukemogenic chromosome translocations. The bottleneck is evidence that leukemia initiation, by in utero ac...Childhood leukemia bottleneck phenomenon is the most mysterious corollary of the prenatal origin discovery of leukemogenic chromosome translocations. The bottleneck is evidence that leukemia initiation, by in utero acquired chromosome translocations that generate functional fusion genes, is far more common than the incidence rate of corresponding leukemia. For childhood TEL-AML1 ^+ acute lymphoblastic leukemia (ALL) this equates to approximately 100 times. Practically this means that among a hundred children born with TEL-AML1 fusion gene, only one child will later in its life develop ALL. The key data necessary for unraveling of this mystery were discovered in 2002. It was the level of TEL-AML1^+cells' frequency. The bottleneck is caused by the very low body TEL-AML1^+cell count. Only one out of a thousand B cells carries TEL-AML1 fusion gene. TEL-AML1^+body cell count is low because TEL-AML1 fusion is generated at cell level of 103 to 104 just during the late fetal lymphopoiesis i.e. after the 36th gestational week.展开更多
Objective: To estimate the relationship between genetic polymorphisms of X-ray repair cross- complementing group 1 (XRCC1) and the susceptibility to childhood acute lymphoblastic leukemia (ALL). Methods: Relevan...Objective: To estimate the relationship between genetic polymorphisms of X-ray repair cross- complementing group 1 (XRCC1) and the susceptibility to childhood acute lymphoblastic leukemia (ALL). Methods: Relevant case-control studies were enrolled in the meta-analysis. We applied Rev Man 4.2 software to pool raw data and test studies' heterogeneity and to calculate the incorporated odds ratio (OR) and 95% confidence interval (95% CI). Results: Our data showed that the OR for the Gln allele of the Arg399Gln polymorphism, compared with the Arg allele, was 1.35 (95% CI, 1.16-1.57; P〈0.0001) for childhood ALL patients. Similarly, the homozygous genotype Gln/Gln and heterozygous genotype Arg/Gln both significantly increased the risk of childhood ALL compared with the wild genotype Arg/Arg (OR =1.58; 95% CI, 1.13-2.21; P=0.008; OR =1.51; 95% CI, 1.21-1.87; P=0.0002). The dominant model of Arg399Gln was associated with childhood ALL risk (OR =1.54; 95% CI, 1.25-1.89; P〈0.0001). The ethnic subgroup analysis demonstrated that the Gln allele in all five ethnic groups was prone to be a risk factor for childhood ALL just with different degrees of correlation while Arg194Trp SNP showed a protective or risk factor or irrelevant thing in different races. Conclusions: XRCC1 399 polymorphism may increase the risk of childhood ALL. Different ethnic groups with some gene polymorphism have different disease risks.展开更多
Pyoderma gangrenosum (PG) is a rare but serious ulcerating skin disease that occurs either idiopathically or associated with various systemic diseases and malignant tumors. Although the association of PG with myeloid ...Pyoderma gangrenosum (PG) is a rare but serious ulcerating skin disease that occurs either idiopathically or associated with various systemic diseases and malignant tumors. Although the association of PG with myeloid malignant tumors is well known, their association with lymphoid malignant tumors, especially in children is extremely rare. We present here a case of PG in a 4-year</span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> and </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">4</span></span></span><span><span><span style="font-family:""> </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">month</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">s</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> female child, </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">diagnosed</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> in the Pediatrics department, which had initially evolved well with oral corticosteroid therapy and a local dermocorticoid. The development was marked 7 months after stopping corticosteroid treatment by the occurrence of acute lymphoblastic leukemia.</span></span></span><span><span><span style="font-family:""> </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">This case of PG is presented in the intention of increasing awareness of this uncommon condition with may oc</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">c</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">ur</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">ring</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> in association with haematological malignancy. PG may precede, develop concurrently with, or follow various conditions</span></span></span><span style="font-family:Verdana;">.展开更多
Background: Having a child with acute lymphoblastic leukemia represents challenges to the family which may impact their quality of life. In such circumstances, parents use various coping strategies to face this stress...Background: Having a child with acute lymphoblastic leukemia represents challenges to the family which may impact their quality of life. In such circumstances, parents use various coping strategies to face this stressful situation, and understanding the relation of coping strategies on quality of life among parents of children with acute lymphoblastic leukemia may provide professionals with ideas about how to support them which in turn may improve their quality of life. Aim: The aim of the present study was to assess the correlation between coping strategies and the health related quality of life of parents whose children are suffering from acute lymphoblastic leukemia. Study design: A cross-sectional design using guided self-administered questionnaire by a single researcher was used. Methods: A universal sample consisted of 299 parents of children with acute lymphoblastic leukemia (ALL) was recruited from Hospital Universiti Kebangsaan Malaysia and Kuala Lumpur Institute Pediatrics. The response rate was 98%. The data collection tools were WHOQOL BREF which is the World Health Organization Quality of Life shorter version and Brief COPE questionnaires. Results: The results revealed that half of the parents perceived their health related quality of life as good. The highest scores for parents were reported on the social relationship domain meanwhile the lowest were reported on the environmental health domain. In regard to the coping strategies, it was found that religion was most frequently used while humor was the least to be used. Moreover, there was a significant positive relationship between quality of life of the parents and problem-focused coping style and a significant negative relationship with emotion-focused coping style. Conclusion: On the basis of the finding of present study, an appropriate intervention could be design to include program that promote the use of problem-focused copings such as active coping and planning and discourage the use of emotion-focused copings such as self-blaming to enhance parents’ health related quality of life.展开更多
Angiogenesis has an important role in pathophysiology of cancer. FMS-like tyrosine kinase 3 (FLT3) is implicated in hematopoietic malignancies. Their role in childhood acute lymphoblastic leukemia (ALL) pathogenesis n...Angiogenesis has an important role in pathophysiology of cancer. FMS-like tyrosine kinase 3 (FLT3) is implicated in hematopoietic malignancies. Their role in childhood acute lymphoblastic leukemia (ALL) pathogenesis needs more enlightenment. Expression of vascular endothelial growth factor receptor-1 and -2 (VEGFR-1 and -2), as well as FLT3 were assessed by flow cytometry in bone marrow (BM) blasts of 55 newly diagnosed children with ALL. Patients included B cell ALL (B-ALL) group (n = 41) and T cell ALL (T-ALL) group (n = 14). Comparison between groups revealed a significant increase in blasts percent (%) expressing FLT3 and FLT3 intensity detected in B-ALL group (p = 0.004 and p = 0.02, respectively). In B-ALL patients, a significant positive correlation was seen between blasts % expressing FLT3 and blasts percentage infiltrating BM (r = 0.405;p = 0.009), also positive correlation was seen between % of blasts expressing VEGFR-1 and VEGFR-2 (r = 0.704;p 0.001). In T-ALL group, blast % expressing FLT3 revealed significant positive correlations with blast % expressing VEGFR-1, and those expressing VEGFR-2 (r = 0.627;p = 0.016, and r = 0.654;p = 0.011, respectively). In addition, significant correlation was seen in blasts % expressing all;FLT3, VEGFR-1 and -2, with blasts % expressing stem cell marker CD34 (r = 0.826;p = 0.001, r = 0.596;p = 0.041, and r = 0.798;p = 0.002, respectively). Conclusion: Expression of VEGFR-1, VEGFR-2 and FLT3 were demonstrated and linked on leukemic blasts of ALL which highlights their role in pathogenesis. FLT3 expression plays a role in facilitating blasts proliferation in BM in B-ALL. FLT3, VEGFR-1 and -2 could be used in future profiling of CD34+ leukemic stem cell pool in T-ALL.展开更多
Background: To lower the mori-mortality related to the aggressiveness of acute leukemia’s chemotherapy regimens, we have implemented in 2017 two low toxicity chemotherapy regimens and new supportive care strategies. ...Background: To lower the mori-mortality related to the aggressiveness of acute leukemia’s chemotherapy regimens, we have implemented in 2017 two low toxicity chemotherapy regimens and new supportive care strategies. The aim of the study is to evaluate our new treatments. Materials and Method: A retrospective study was carried out from January 2014 to May 2021 in the hematology department of the teaching hospital in Brazzaville. The study concerned 47 children diagnosed with acute leukemia. Participants were divided into two groups: acute leukemia diagnosed before 2017 (group 1: 23 children) and after 2017 (group 2: 14 children). They were compared using the chi-square. Results: The median age was 10.0 ± 5.01 years. Features and outcomes of group 2 were better. The median duration of symptoms was shorter: 2.45 ± 2.87 months (p = 0.036). The Karnosky score was higher (p = 0.002) and white blood cell count lower (p = 0.331). Both groups started the treatment with a delay of 6 days. The induction treatment was completed in 69.6% before 2017 versus 93.3% after. The rate of relapse was more important for group 1: 85.7% versus 14.3% (p = 0.01). Conclusion: Trainings of professionals have improved the characteristics outcomes of our patients and should be pursued. Considering the high relapse rate, our protocol will need to be intensified.展开更多
文摘Childhood leukemia bottleneck phenomenon is the most mysterious corollary of the prenatal origin discovery of leukemogenic chromosome translocations. The bottleneck is evidence that leukemia initiation, by in utero acquired chromosome translocations that generate functional fusion genes, is far more common than the incidence rate of corresponding leukemia. For childhood TEL-AML1 ^+ acute lymphoblastic leukemia (ALL) this equates to approximately 100 times. Practically this means that among a hundred children born with TEL-AML1 fusion gene, only one child will later in its life develop ALL. The key data necessary for unraveling of this mystery were discovered in 2002. It was the level of TEL-AML1^+cells' frequency. The bottleneck is caused by the very low body TEL-AML1^+cell count. Only one out of a thousand B cells carries TEL-AML1 fusion gene. TEL-AML1^+body cell count is low because TEL-AML1 fusion is generated at cell level of 103 to 104 just during the late fetal lymphopoiesis i.e. after the 36th gestational week.
文摘Objective: To estimate the relationship between genetic polymorphisms of X-ray repair cross- complementing group 1 (XRCC1) and the susceptibility to childhood acute lymphoblastic leukemia (ALL). Methods: Relevant case-control studies were enrolled in the meta-analysis. We applied Rev Man 4.2 software to pool raw data and test studies' heterogeneity and to calculate the incorporated odds ratio (OR) and 95% confidence interval (95% CI). Results: Our data showed that the OR for the Gln allele of the Arg399Gln polymorphism, compared with the Arg allele, was 1.35 (95% CI, 1.16-1.57; P〈0.0001) for childhood ALL patients. Similarly, the homozygous genotype Gln/Gln and heterozygous genotype Arg/Gln both significantly increased the risk of childhood ALL compared with the wild genotype Arg/Arg (OR =1.58; 95% CI, 1.13-2.21; P=0.008; OR =1.51; 95% CI, 1.21-1.87; P=0.0002). The dominant model of Arg399Gln was associated with childhood ALL risk (OR =1.54; 95% CI, 1.25-1.89; P〈0.0001). The ethnic subgroup analysis demonstrated that the Gln allele in all five ethnic groups was prone to be a risk factor for childhood ALL just with different degrees of correlation while Arg194Trp SNP showed a protective or risk factor or irrelevant thing in different races. Conclusions: XRCC1 399 polymorphism may increase the risk of childhood ALL. Different ethnic groups with some gene polymorphism have different disease risks.
文摘Pyoderma gangrenosum (PG) is a rare but serious ulcerating skin disease that occurs either idiopathically or associated with various systemic diseases and malignant tumors. Although the association of PG with myeloid malignant tumors is well known, their association with lymphoid malignant tumors, especially in children is extremely rare. We present here a case of PG in a 4-year</span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> and </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">4</span></span></span><span><span><span style="font-family:""> </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">month</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">s</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> female child, </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">diagnosed</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> in the Pediatrics department, which had initially evolved well with oral corticosteroid therapy and a local dermocorticoid. The development was marked 7 months after stopping corticosteroid treatment by the occurrence of acute lymphoblastic leukemia.</span></span></span><span><span><span style="font-family:""> </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">This case of PG is presented in the intention of increasing awareness of this uncommon condition with may oc</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">c</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">ur</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">ring</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> in association with haematological malignancy. PG may precede, develop concurrently with, or follow various conditions</span></span></span><span style="font-family:Verdana;">.
文摘Background: Having a child with acute lymphoblastic leukemia represents challenges to the family which may impact their quality of life. In such circumstances, parents use various coping strategies to face this stressful situation, and understanding the relation of coping strategies on quality of life among parents of children with acute lymphoblastic leukemia may provide professionals with ideas about how to support them which in turn may improve their quality of life. Aim: The aim of the present study was to assess the correlation between coping strategies and the health related quality of life of parents whose children are suffering from acute lymphoblastic leukemia. Study design: A cross-sectional design using guided self-administered questionnaire by a single researcher was used. Methods: A universal sample consisted of 299 parents of children with acute lymphoblastic leukemia (ALL) was recruited from Hospital Universiti Kebangsaan Malaysia and Kuala Lumpur Institute Pediatrics. The response rate was 98%. The data collection tools were WHOQOL BREF which is the World Health Organization Quality of Life shorter version and Brief COPE questionnaires. Results: The results revealed that half of the parents perceived their health related quality of life as good. The highest scores for parents were reported on the social relationship domain meanwhile the lowest were reported on the environmental health domain. In regard to the coping strategies, it was found that religion was most frequently used while humor was the least to be used. Moreover, there was a significant positive relationship between quality of life of the parents and problem-focused coping style and a significant negative relationship with emotion-focused coping style. Conclusion: On the basis of the finding of present study, an appropriate intervention could be design to include program that promote the use of problem-focused copings such as active coping and planning and discourage the use of emotion-focused copings such as self-blaming to enhance parents’ health related quality of life.
文摘Angiogenesis has an important role in pathophysiology of cancer. FMS-like tyrosine kinase 3 (FLT3) is implicated in hematopoietic malignancies. Their role in childhood acute lymphoblastic leukemia (ALL) pathogenesis needs more enlightenment. Expression of vascular endothelial growth factor receptor-1 and -2 (VEGFR-1 and -2), as well as FLT3 were assessed by flow cytometry in bone marrow (BM) blasts of 55 newly diagnosed children with ALL. Patients included B cell ALL (B-ALL) group (n = 41) and T cell ALL (T-ALL) group (n = 14). Comparison between groups revealed a significant increase in blasts percent (%) expressing FLT3 and FLT3 intensity detected in B-ALL group (p = 0.004 and p = 0.02, respectively). In B-ALL patients, a significant positive correlation was seen between blasts % expressing FLT3 and blasts percentage infiltrating BM (r = 0.405;p = 0.009), also positive correlation was seen between % of blasts expressing VEGFR-1 and VEGFR-2 (r = 0.704;p 0.001). In T-ALL group, blast % expressing FLT3 revealed significant positive correlations with blast % expressing VEGFR-1, and those expressing VEGFR-2 (r = 0.627;p = 0.016, and r = 0.654;p = 0.011, respectively). In addition, significant correlation was seen in blasts % expressing all;FLT3, VEGFR-1 and -2, with blasts % expressing stem cell marker CD34 (r = 0.826;p = 0.001, r = 0.596;p = 0.041, and r = 0.798;p = 0.002, respectively). Conclusion: Expression of VEGFR-1, VEGFR-2 and FLT3 were demonstrated and linked on leukemic blasts of ALL which highlights their role in pathogenesis. FLT3 expression plays a role in facilitating blasts proliferation in BM in B-ALL. FLT3, VEGFR-1 and -2 could be used in future profiling of CD34+ leukemic stem cell pool in T-ALL.
文摘Background: To lower the mori-mortality related to the aggressiveness of acute leukemia’s chemotherapy regimens, we have implemented in 2017 two low toxicity chemotherapy regimens and new supportive care strategies. The aim of the study is to evaluate our new treatments. Materials and Method: A retrospective study was carried out from January 2014 to May 2021 in the hematology department of the teaching hospital in Brazzaville. The study concerned 47 children diagnosed with acute leukemia. Participants were divided into two groups: acute leukemia diagnosed before 2017 (group 1: 23 children) and after 2017 (group 2: 14 children). They were compared using the chi-square. Results: The median age was 10.0 ± 5.01 years. Features and outcomes of group 2 were better. The median duration of symptoms was shorter: 2.45 ± 2.87 months (p = 0.036). The Karnosky score was higher (p = 0.002) and white blood cell count lower (p = 0.331). Both groups started the treatment with a delay of 6 days. The induction treatment was completed in 69.6% before 2017 versus 93.3% after. The rate of relapse was more important for group 1: 85.7% versus 14.3% (p = 0.01). Conclusion: Trainings of professionals have improved the characteristics outcomes of our patients and should be pursued. Considering the high relapse rate, our protocol will need to be intensified.