Quantification of granulocyte-macrophage colony-stimulating factor hypersensitivity in juvenile myelomonocytic leukemia by ³H-thymidine assay

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• 作者：Makito Tanaka ; Yoshiyuki Takahashi ; YinYan Xu ; Nao Yoshida ; Ayami Yoshimi ; Itzel Bustos Villalobos ; Asahito Hama ; Nobuhiro Nishio ; Hirokazu Hidaka ; Yue Wang ; Hiroshi Yagasaki ; Seiji Kojima
• 关键词：Juvenile myelomonocytic leukemia (JMML) ; Colony-forming unit granulocyte-macrophage (CFU-GM) ; Granulocyte-macrophage colony-stimulating factor (GM-CSF) ; Hypersensitivity ; ³H-thymidine assay ; RAS mutation ; PTPN11 mutation
• 刊名：Leukemia Research
• 出版年：2008
• 期刊代码：186_01452126
• 类别：med
• 出版时间：July 2008
• 卷：32
• 期：7
• 页码：1036-1042
• 文件大小：515 K

摘要

Recent molecular studies have revealed that the GM-CSF/RAS signaling pathway plays a central role in the pathogenesis of juvenile myelomonocytic leukemia (JMML). CFU-GM colony assay is an important test for GM-CSF hypersensitivity in patients with JMML, but requires specific skills. We established a simple and easy quantification method to test GM-CSF hypersensitivity, using a ³H-thymidine assay. With this quantification method, JMML patients with RAS mutations showed significantly higher GM-CSF sensitivity than JMML patients with PTPN11 mutations. This method will be useful not only in the diagnosis of JMML, but also to evaluate the difference of GM-CSF sensitivity among patients.