Clinical Courses of Two Pediatric Patients with Acute Megakaryoblastic Leukemia Harboring the CBFA2T3-GLIS2 Fusion Gene [Turk J Hematol]
Turk J Hematol. 2016; 33(4): 331-334 | DOI: 10.4274/tjh.2016.0008  

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

Mayu Ishibashi1, Tomoko Yokosuka2, Masakatsu Yanagimachi1, Fuminori Iwasaki3, Shin-ichi Tsujimoto1, Koji Sasaki1, Masanobu Takeuchi1, Reo Tanoshima1, Hiromi Kato1, Ryosuke Kajiwara1, Fumiko Tanaka1, Hiroaki Goto2, Shumpei Yokota1
1Yokohama City University Faculty of Medicine, Department of Pediatrics, Yokohama, Japan
2Yokohama City University Faculty of Medicine, Department of Pediatrics, Yokohama, Japan, Kanagawa Children’s Medical Center, Clinic of Hematology/Oncology and Regenerative Medicine, Yokohama, Japan
3Kanagawa Children’s Medical Center, Clinic of Hematology/Oncology and Regenerative Medicine, Yokohama, Japan

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.

Keywords: Acute megakaryoblastic leukemia without Down syndrome, CBFA2T3-GLIS2 fusion gene


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

Mayu Ishibashi1, Tomoko Yokosuka2, Masakatsu Yanagimachi1, Fuminori Iwasaki3, Shin-ichi Tsujimoto1, Koji Sasaki1, Masanobu Takeuchi1, Reo Tanoshima1, Hiromi Kato1, Ryosuke Kajiwara1, Fumiko Tanaka1, Hiroaki Goto2, Shumpei Yokota1
1Yokohama City University Faculty of Medicine, Department of Pediatrics, Yokohama, Japan
2Yokohama City University Faculty of Medicine, Department of Pediatrics, Yokohama, Japan, Kanagawa Children’s Medical Center, Clinic of Hematology/Oncology and Regenerative Medicine, Yokohama, Japan
3Kanagawa Children’s Medical Center, Clinic of Hematology/Oncology and Regenerative Medicine, Yokohama, Japan

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.

Anahtar Kelimeler: Down sendromu olmayanlarda akut megakaryoblastik lösemi, CBFA2T3-GLIS2 füzyon geni


Mayu Ishibashi, Tomoko Yokosuka, Masakatsu Yanagimachi, Fuminori Iwasaki, Shin-ichi Tsujimoto, Koji Sasaki, Masanobu Takeuchi, Reo Tanoshima, Hiromi Kato, Ryosuke Kajiwara, Fumiko Tanaka, Hiroaki Goto, Shumpei Yokota. Clinical Courses of Two Pediatric Patients with Acute Megakaryoblastic Leukemia Harboring the CBFA2T3-GLIS2 Fusion Gene. Turk J Hematol. 2016; 33(4): 331-334

Corresponding Author: Masakatsu Yanagimachi, Japan


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