Pediatric Myelodysplastic Syndromes: They Do Exist!
Pediatric Myelodysplastic Syndromes: They Do Exist!
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DOI：
10.1097/mph.0000000000000046
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发表时间：
2014-01-01
影响因子：
1.2
通讯作者：
Corey, Seth J.
中科院分区：
文献类型：
--
作者：
Glaubach, Taly;Robinson, Lisa J.;Corey, Seth J.
骨髓增生异常综合征(MDS)是成人最常见的血液系统恶性肿瘤之一，是一组异质性克隆性疾病，以外周血细胞减少(S)和正常或高细胞骨髓伴>；=1血细胞系异常增生为特征。MDS易发展为继发性急性髓系白血病，预后差。虽然在儿童恶性血液病中不常见，但初发和继发性MDS都发生在儿童中，可能是遗传性骨髓衰竭综合征的第一个表现。与成人MDS不同，儿童MDS更常伴有骨髓细胞减少和单体7。难治性红细胞减少症比难治性贫血更典型，如在老年人中所见。它的识别和管理可能是相当具有挑战性的，需要有经验的血液病理学家的专业知识。在这篇综述中，我们描述了儿童MDS的流行病学、遗传学和临床谱，以及它的诊断和治疗挑战。我们还比较和对比了儿童和成人的MDS。
One of the most common hematologic malignancies in adults, myelodysplastic syndrome (MDS) is a heterogenous group of clonal disorders characterized by peripheral cytopenia(s) and normal or hypercellular bone marrow with dysplasia in >= 1 blood cell lineages. MDS frequently evolves to secondary acute myeloid leukemia with poor prognosis. Although uncommon among pediatric hematologic malignancies, both de novo and secondary MDS occur in children and may be the first presentation of an inherited bone marrow failure syndrome. Unlike its adult counterpart, pediatric MDS is more frequently associated with hypocellular bone marrow and monosomy 7. Refractory cytopenia is more typical than refractory anemia, as seen in the elderly. Its recognition and management can be quite challenging and requires the expertise of an experienced hematopathologist. In this review, we describe the epidemiology, genetics, and clinical spectrum of pediatric MDS along with its diagnostic and therapeutic challenges. We also compare and contrast pediatric and adult MDS.