Journals / Turkish Journal of Hematology / 2016 / Cilt: 33 - Sayı: 4

CBFA2T3-GLIS2 Füzyon Geni Taşıyan İki Pediatrik Akut Megakaryoblastik Lösemi Hastasının Klinik Seyri

Clinical Courses of Two Pediatric Patients with Acute Megakaryoblastic Leukemia Harboring the CBFA2T3-GLIS2 Fusion Gene

Pages
331–334
DOI
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AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

We report the difficult clinical courses of pediatric patients with AMKL harboring the CBFA2T3-GLIS2 fusion gene.

📌 3 Key Takeaways

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Objective & Hypothesis:Acute megakaryoblastic leukemia (AMKL) in children without Down syndrome (DS) has an extremely poor outcome with 3-year survival of less than 40%, whereas AMKL in children with DS has an excellent survival rate.
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Key Findings & Data:Recently, a novel recurrent translocation involving CBFA2T3 and GLIS2 was identified in about 30% of children with non-DS AMKL, and the fusion gene was reported as a strong poor prognostic factor in pediatric AMKL.
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Conclusion & Impact:We report the difficult clinical courses of pediatric patients with AMKL harboring the CBFA2T3-GLIS2 fusion gene.
🔬Design:Research Article
📍Scope:Comprehensive Sample
⚙️Variables:Domain Parameters

Abstract

Acute megakaryoblastic leukemia (AMKL) in children without Down syndrome (DS) has an extremely poor outcome with 3-year survival of less than 40%, whereas AMKL in children with DS has an excellent survival rate. Recently, a novel recurrent translocation involving CBFA2T3 and GLIS2 was identified in about 30% of children with non-DS AMKL, and the fusion gene was reported as a strong poor prognostic factor in pediatric AMKL. We report the difficult clinical courses of pediatric patients with AMKL harboring the CBFA2T3-GLIS2 fusion gene.

Özet

Down sendromu (DS) olmayan çocuklarda akut megakaryoblastik löseminin (AMKL) prognozu çok kötü ve 3 yıllık sağkalım %40'ın altında iken, DS'li çocuklarda AMKL'nin sağkalım oranı mükemmeldir. Yakın zamanda, DS olmayan AMKL'li çocukların yaklaşık %30'unda CBFA2T3 ve GLIS2'yi içeren yeni bir tekrarlayan translokasyon tanımlandı ve füzyon geninin pediatrik AMKL olgularında kötü prognoz ile ilişkili güçlü bir prognostik belirteç olduğu bildirildi. CBFA2T3-GLIS2 füzyon genini taşıyan AMKL tanılı pediatrik hastalarda sorunlu klinik seyri bildiriyoruz.

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Cite This Article

ISHİBASHİ, M., YOKOSUKA, T., YANAGİMACHİ, M. D., IWASAKİ, F., TSUJİMOTO, S., SASAKİ, K., TAKEUCHİ, M., TANOSHİMA, R., KATO, H., KAJİWARA, R., TANAKA, F., GOTO, H., YOKOTA, S. (2016). CBFA2T3-GLIS2 Füzyon Geni Taşıyan İki Pediatrik Akut Megakaryoblastik Lösemi Hastasının Klinik Seyri. Turkish Journal of Hematology, Cilt: 33 - Sayı: 4, 331–334. https://ajindex.com/articles/412493

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