دورية أكاديمية
Treatment of acute leukemia in children with ataxia telangiectasia (A-T).
| العنوان: | Treatment of acute leukemia in children with ataxia telangiectasia (A-T). |
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| المؤلفون: | Schoenaker MH; Department of Pediatrics, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, Netherlands., Suarez F; Department of Hematology and French National Reference Center for Primary Immune Deficiencies (CEREDIH), Necker-Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Laboratory of Molecular Mechanisms of Hematologic Disorders and Therapeutic Implication, INSERM UMR 1163 & CNRS ERL 8254, France; Institut Imagine, Sorbonne Paris Cité, Paris Descartes University, France., Szczepanski T; Department of Pediatric Hematology and Oncology, Zabrze, Medical University of Silesia, Katowice, Poland., Mahlaoui N; Pediatric Immuno-Haematology and Rheumatology Unit and French National Reference Center for Primary Immune Deficiencies (CEREDIH), Necker-Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Paris Descartes University, Sorbonne Paris Cité, Imagine Institute, Paris, France., Loeffen JL; Department of Pediatric Oncology and Hematology, Sophia Children's Hospital, Erasmus MC, Postbox 2040, 3000CA Rotterdam, Netherlands. Electronic address: j.loeffen@erasmusmc.nl. |
| المصدر: | European journal of medical genetics [Eur J Med Genet] 2016 Dec; Vol. 59 (12), pp. 641-646. Date of Electronic Publication: 2016 May 27. |
| نوع المنشور: | Journal Article; Meta-Analysis |
| اللغة: | English |
| بيانات الدورية: | Publisher: Elsevier Country of Publication: Netherlands NLM ID: 101247089 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1878-0849 (Electronic) Linking ISSN: 17697212 NLM ISO Abbreviation: Eur J Med Genet Subsets: MEDLINE |
| أسماء مطبوعة: | Original Publication: Amsterdam : Elsevier, c2005- |
| مواضيع طبية MeSH: | Ataxia Telangiectasia/*drug therapy , Ataxia Telangiectasia/*epidemiology , Precursor T-Cell Lymphoblastic Leukemia-Lymphoma/*drug therapy , Precursor T-Cell Lymphoblastic Leukemia-Lymphoma/*epidemiology, Ataxia Telangiectasia/complications ; Ataxia Telangiectasia/pathology ; Child ; Disease-Free Survival ; Humans ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma/complications ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma/pathology ; Prospective Studies |
| مستخلص: | Early onset ataxia telangiectasia (A-T) is a neurodegenerative DNA-instability disorder, which presents early in childhood. Hallmarks of A-T are progressive ataxia and a dramatic increased risk of developing malignancies (25%), especially of hematological origin. In children these malignancies mainly concern aggressive Non-Hodgkin lymphoma, acute leukemias and Hodgkin lymphoma. Of the acute leukemias, T-cell lymphoblastic leukemia (T-ALL) is by far the most common. Since patients with A-T experience increased toxicity to radio- and chemotherapeutic treatment, the optimal treatment strategy of acute leukemia remains subject of debate. Review of literature of treatment of T-ALL in patients with A-T (n = 18) showed that many patients are not diagnosed with A-T at time of presentation of T-ALL. This implicates that physicians must be aware of symptoms of A-T in young patients presenting with T-ALL. Complete remission rates are high following upfront modified as well as unmodified treatment strategies. Treatment of ALL in children with A-T is feasible and should be performed. Definitive treatment strategy must be determined by shared decision making with patient, caretakers and medical team. Future prospective studies are needed to elucidate optimal treatment strategy. (Copyright © 2016. Published by Elsevier Masson SAS.) |
| فهرسة مساهمة: | Keywords: Ataxia telangiectasia; Cancer; Children; Leukemia; Malignancy |
| تواريخ الأحداث: | Date Created: 20160531 Date Completed: 20170210 Latest Revision: 20170210 |
| رمز التحديث: | 20231215 |
| DOI: | 10.1016/j.ejmg.2016.05.012 |
| PMID: | 27238889 |
| قاعدة البيانات: | MEDLINE |
Full Text via ClinicalKey 
Full Text Finder | تدمد: | 1878-0849 |
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| DOI: | 10.1016/j.ejmg.2016.05.012 |