Acute Leukemias in Children with Down Syndrome

Zwaan, C. Michel; Reinhardt, Dirk; Vyas, Paresh

doi:10.1016/j.pcl.2007.11.001

Cited by 58 publications

(39 citation statements)

References 75 publications

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“…The leukemic cells of patients with Down syndrome are usually highly sensitive to chemotherapy with an exceptional high survival rate, and therefore it is possible to treat these patients with adjusted treatment protocols [9]. In addition, AML may occur following previous radiotherapy or chemotherapy containing alkylating agents or epipodophyllotoxins, as secondary neoplasm.…”

Section: Clinical Introductionmentioning

confidence: 99%

“…In contrast, poor survival rates were reported in pediatric AML with translocations t(6;11)(q27;q23) and t(10;11)(p12;q23) [80,81]. The acute megakaryoblastic leukemias (AMKL, FABM7) in non-Down syndrome patients represent a subgroup with poor outcome, with the exception of AMKL harboring t(1;22)(p13;q13), which seems to confer a favorable prognostic group, in contrast to Down syndrome, where AMKL confers a favorable outcome [9,17]. Monosomy 7 is a well-known poor-prognostic factor and confers a worse outcome [52,82].…”

Section: Clinical Introductionmentioning

confidence: 99%

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Pediatric AML: From Biology to Clinical Management

Rooij

2015

JCM

174

169

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Pediatric acute myeloid leukemia (AML) represents 15%–20% of all pediatric acute leukemias. Survival rates have increased over the past few decades to ~70%, due to improved supportive care, optimized risk stratification and intensified chemotherapy. In most children, AML presents as a de novo entity, but in a minority, it is a secondary malignancy. The diagnostic classification of pediatric AML includes a combination of morphology, cytochemistry, immunophenotyping and molecular genetics. Outcome is mainly dependent on the initial response to treatment and molecular and cytogenetic aberrations. Treatment consists of a combination of intensive anthracycline- and cytarabine-containing chemotherapy and stem cell transplantation in selected genetic high-risk cases or slow responders. In general, ~30% of all pediatric AML patients will suffer from relapse, whereas 5%–10% of the patients will die due to disease complications or the side-effects of the treatment. Targeted therapy may enhance anti-leukemic efficacy and minimize treatment-related morbidity and mortality, but requires detailed knowledge of the genetic abnormalities and aberrant pathways involved in leukemogenesis. These efforts towards future personalized therapy in a rare disease, such as pediatric AML, require intensive international collaboration in order to enhance the survival rates of pediatric AML, while aiming to reduce long-term toxicity.

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Section: Clinical Introductionmentioning

confidence: 99%

Section: Clinical Introductionmentioning

confidence: 99%

Pediatric AML: From Biology to Clinical Management

Rooij

2015

JCM

174

169

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“…In this study, Down syndrome was reported as one of the risk factors for ALL in children. The role of Down syndrome (trisomy 21), as a risk factor for the incidence of ALL children, has been shown in numerous studies, in which children with Down syndrome have a significantly higher risk of ALL as compared to children without it (18)(19)(20)(21)(22).…”

Section: Discussionmentioning

confidence: 99%

Pre- and Post- birth Causes of Acute Lymphoblastic Leukemia

et al. 2018

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Background: Acute lymphoblastic leukemia (ALL) is a malignancy of lymphoid progenitors in bone marrow, peripheral blood, and extra medullar, which accounts for 1/4 of childhood cancers. Objectives: Regarding the uncertainties of the exact causes of the disease and the importance of identifying risk factors of acute lymphoblastic leukemia, this study aimed at investigating the causes of pre-and post -natal birth in children with ALL. Methods: This case -control study was performed on 156 patients with ALL and 85 patients without ALL in a 4 -year period in Ali Asghar, Shahid Faghihi, and Shahid Motahari Hospitals in Shiraz, Iran between January 2013 and March 2017. The student's t and Chi -square tests were used. To evaluate relationships between various variables, Pearson's or Spearman's correlation analyses were used. The data were analyzed, using the SPSS 16 software. Results: Based on the findings of this study, the presence of Dawn's syndrome and familial history of leukemia and brain tumors were identified as risk factors for the incidence of ALL in children. There was no significant relationship with the history of abortion, radiation exposure, economic status, place of residence, birth rate, etc. Conclusions:The results indicate that among pre-and post -birth causes, the presence of Down syndrome and familial history of leukemia and brain tumors were as risk factors for the incidence of ALL in children. Due to the fact that therapeutic protocols are useful for the treatment of ALL in children, the results of this study and similar studies can be effective in preventing and managing the disease in children.

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“…The European BFM trial utilizes a standard BFM regimen with cytarabine and anthracycline dose reductions. [37] The U.S. trial, open only to DS children under four years, features high-dose cytarabine, reduced anthracycline, and reduced intrathecal therapy. Both trials eliminate maintenance therapy, cranial irradiation, and stem cell transplantation.…”

Section: Myeloid Disorders In Dsmentioning

confidence: 99%

Malignancy in Children with Trisomy 21

Rabin

Whitlock

2009

The Oncologist

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The content of this article has been reviewed by independent peer reviewers to ensure that it is balanced, objective, and free from commercial bias. Target audience:Physicians who wish to advance their current knowledge of clinical cancer medicine in pediatric oncology. LEARNING OBJECTIVES1. Evaluate malignancies for which children with Down syndrome are at increased and decreased risk in order to screen appropriately.2. Analyze the clinical and biologic features of transient myeloproliferative disease and acute megakaryoblastic leukemia in children with DS.3. Determine the clinical and biologic features of acute lymphoblastic leukemia in children with DS and outline treatment strategies.This article is available for continuing medical education credit at CME.TheOncologist.com. CME CME ABSTRACTPatients with Down syndrome (DS) display a unique spectrum of malignancies, with a 10-to 20-fold higher risk of acute leukemias, and a markedly lower incidence of solid tumors. This review discusses the current understanding of the basis for this distinctive pattern of cancer incidence and the clinical and biologic features of the malignant disorders most frequent in DS individuals: transient myeloproliferative disease, acute megakaryoblastic leukemia, and acute lymphoblastic leukemia. We also review distinctive pharmacogenetic issues, highlighting the differential chemosensitivity and toxicity profiles of DS patients compared with the general population, and epidemiologic studies of protective and adverse environmental risk factors for the development of leukemia.

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Acute Leukemias in Children with Down Syndrome

Cited by 58 publications

References 75 publications

Pediatric AML: From Biology to Clinical Management

Pediatric AML: From Biology to Clinical Management

Pre- and Post- birth Causes of Acute Lymphoblastic Leukemia

Malignancy in Children with Trisomy 21

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