Adachi Souichi, Manabe Atsushi, Imaizumi Masue, Taga Takashi, Tawa Akio, Tsurusawa Masahito, Kikuchi Akira, Masunaga Atsuko, Tsuchida Masahiro, Nakahata Tatsutoshi
Department of Pediatrics, Kyoto University Graduate School of Medicine, Kyoto, Japan.
Int J Hematol. 2007 Nov;86(4):358-63. doi: 10.1532/IJH97.07025.
We retrospectively surveyed pediatric acute myeloid leukemia (AML) patients with multilineage dysplasia treated with the AML 99 and the Children's Cancer and Leukemia Study Group (CCLSG) AML 9805 protocols. We found only 9 AML patients (2.6%) with multilineage dysplasia among the 341 patients with newly diagnosed de novo AML. Eight of the 9 patients obtained complete remission (CR) following the intensive AML-oriented treatments. Three of 7 patients who underwent stem cell transplantation were alive in CR for more than 4 years, and the 2 patients treated only with chemotherapy were alive in CR for more than 30 months. We did not identify any particular chromosomal abnormalities or differentiation according to the French-American-British classification in these 9 patients. No reports have described AML with multilineage dysplasia in children, and the incidence of the disease is expected to be very low. We plan to conduct a prospective pathologic review to select cases with this disease entity in the next Japanese Pediatric Leukemia/Lymphoma Study Group (JPLSG) AML-05 protocol.
我们回顾性调查了采用AML 99方案以及儿童癌症与白血病研究组(CCLSG)AML 9805方案治疗的伴有多系发育异常的小儿急性髓系白血病(AML)患者。在341例新诊断的原发性AML患者中,我们仅发现9例(2.6%)伴有多系发育异常的AML患者。9例患者中有8例在接受以AML为主的强化治疗后获得完全缓解(CR)。7例接受干细胞移植的患者中有3例在CR状态下存活超过4年,2例仅接受化疗的患者在CR状态下存活超过30个月。在这9例患者中,根据法美英分类法,我们未发现任何特定的染色体异常或分化情况。尚无关于儿童多系发育异常AML的报道,预计该疾病的发病率非常低。我们计划在接下来的日本小儿白血病/淋巴瘤研究组(JPLSG)AML-05方案中进行前瞻性病理检查,以筛选出患有该疾病实体的病例。