Clinical course of juvenile myelomonocytic leukemia in the blast crisis phase treated by acute myeloid leukemia-oriented chemotherapy and allogeneic hematopoietic stem cell transplantation.
Clinical course of juvenile myelomonocytic leukemia in the blast crisis phase treated by acute myeloid leukemia-oriented chemotherapy and allogeneic hematopoietic stem cell transplantation.
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急性髓系白血病定向化疗联合异基因造血干细胞移植治疗急变期幼年粒单核细胞白血病的临床过程。
DOI：
10.1007/s12185-014-1638-3
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发表时间：
2014
期刊：
影响因子：
--
通讯作者：
Takemura T.
中科院分区：
文献类型：
--
作者：
Ueda S;Sakata N;Muramatsu H;Sakaguchi H;Wang X;Xu Y;Kojima S;Yamaguchi T;Higa T;Takemura T.
幼年型粒单核细胞白血病 (JMML) 是一种发生于儿童早期的混合性骨髓增殖性和骨髓增生异常性疾病。 JMML 的临床病程变化很大。三分之一的患者病程相对惰性，但大约 15% 的病例被认为会发展为急性髓系白血病（称为急变危象）。急变的病因和临床特征尚不清楚。我们记录了一名 27 个月大男孩的病例，该男孩出现肝脾肿大、皮疹和淋巴结肿大。急性红系白血病的初步诊断是根据法国-美国-英国分类进行的。经过对 GM-CSF 过敏的评估和 PTPN11 的基因分析后，他被诊断为处于急变期的 JMML。尽管他有几个不良的预后因素，包括7号单体和高HbF百分比，但在接受急性髓系白血病化疗和同种异体造血干细胞移植后，他获得了部分缓解。他已经完全缓解了六年多。
Juvenile myelomonocytic leukemia (JMML) is a mixed myeloproliferative and myelodysplastic disorder that occurs in early childhood. The clinical course of JMML is highly variable. A third of patients follow a relatively indolent course, although approximately 15 % cases are thought to develop acute myeloid leukemia, referred to as blast crisis. The etiology and clinical characteristics of blast crisis remain unclear. We document the case of a 27-month-old boy who presented with hepatosplenomegaly, skin rash, and lymphadenopathy. An initial diagnosis of acute erythroid leukemia was made according to the French–American–British classification. Following estimation of hypersensitivity to GM-CSF and genetic analysis ofPTPN11, he was diagnosed with JMML in the blast crisis phase. Although he had several poor prognostic factors, including monosomy 7 and high HbF percentage, he achieved partial remission after treatment with acute myeloid leukemia-oriented chemotherapy followed by allogeneic hematopoietic stem cell transplantation. He has been in complete remission for over 6 years.