伴有t(7;11)(p15;p15)和NUP98-HOXA11融合的青少年粒单核细胞白血病。
Juvenile myelomonocytic leukemia with t(7;11)(p15;p15) and NUP98-HOXA11 fusion.
作者信息
Mizoguchi Yoko, Fujita Naoto, Taki Tomohiko, Hayashi Yasuhide, Hamamoto Kazuko
机构信息
Department of Pediatrics, Hiroshima Red Cross Hospital and Atomic Bomb Survivors Hospital, Hiroshima, Japan.
出版信息
Am J Hematol. 2009 May;84(5):295-7. doi: 10.1002/ajh.21373.
The t(7;11)(p15;p15) translocation has been reported as a rare and recurrent chromosomal abnormality in acute myeloid leukemia (AML) patients. The NUP98-HOXA9 fusion gene with t(7;11)(p15;p15) was identified and revealed to be essential for leukemogenesis and myeloproliferative disease. To date, t(7;11)(p15;p15) with NUP98-HOXA11 fusion has been reported only in one case of ph-negative chronic myeloid leukemia (CML). Here, we report a case of a 3-year-old girl with juvenile myelomonocytic leukemia (JMML) carrying t(7;11)(p15;p15) abnormality with NUP98-HOXA11 fusion. AML chemotherapy followed by bone marrow transplantation (BMT) was found to be effective in treating this disorder, and she remains in complete remission for 3 years after BMT. We suggest the possibility that AML chemotherapy might be effective for treating JMML with t(7;11)(p15;p15) abnormality and NUP98-HOXA11 fusion.
t(7;11)(p15;p15)易位已被报道为急性髓系白血病（AML）患者中一种罕见且反复出现的染色体异常。具有t(7;11)(p15;p15)的NUP98-HOXA9融合基因已被鉴定，并且被发现对白血病发生和骨髓增殖性疾病至关重要。迄今为止，具有NUP98-HOXA11融合的t(7;11)(p15;p15)仅在1例Ph阴性慢性髓系白血病（CML）中被报道。在此，我们报告1例患有青少年粒单核细胞白血病（JMML）的3岁女孩，其携带具有NUP98-HOXA11融合的t(7;11)(p15;p15)异常。发现AML化疗后进行骨髓移植（BMT）对治疗该疾病有效，并且她在BMT后已完全缓解3年。我们提出AML化疗可能对治疗具有t(7;11)(p15;p15)异常和NUP98-HOXA11融合的JMML有效的可能性。
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