Extramedullary hematopoiesis involving the central nervous system and surrounding structures

Extramedullary hematopoiesis involving the central nervous system and surrounding structures
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DOI:
10.1016/j.humpath.2011.01.006
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发表时间:
2011-10-01
期刊:
影响因子:
3.3
通讯作者:
Del Bigio, Marc R.
Del Bigio, Marc R.
中科院分区:
医学3区
文献类型:
--
作者:
Zherebitskiy, Viktor;Morales, Carmen;Del Bigio, Marc R.

文献摘要

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髓外造血在中枢神经系统是一种罕见的条件,可以出现与血液学条件。在1988年至2010年的单中心回顾性研究中,我们发现了8例涉及中枢神经系统或脑膜的髓外造血。大多数是偶然的尸检发现,通常是显微镜下收集的脑膜或血管周围的细胞在婴儿(4例,年龄,10天至5个月),1名幼儿(年龄,6.5岁,贫血)和1名老年人(年龄,81岁,终末期特发性骨髓纤维化)。2例患者(均患有真性红细胞增多症)出现颅内或椎管病变症状(年龄分别为67岁和68岁)。未成熟造血细胞混合群体通过特征形态学和适当的免疫组织化学鉴定。中枢神经系统髓外造血的确切发病机制尚不清楚。在婴儿中,髓外造血不太可能是病理性的,很可能代表胎儿状态的解决。伴有偶发或症状性髓外造血的大龄儿童和成人通常有一些潜在的血液学疾病,如果尚未被发现,则应寻求治疗。(C) 2011爱思唯尔公司版权所有。
Extramedullary hematopoiesis in central nervous system is a rare condition that can present in association with hematologic conditions. In this single-center retrospective study from 1988 to 2010, we identified 8 cases of extramedullary hematopoiesis involving the central nervous system or meninges. Most were incidental autopsy findings, usually microscopic collections of cells in the meninges or around blood vessels in the brain of infants (4 cases; age, 10 days to 5 months), in 1 young child (age, 6.5 years with anemia), and 1 elderly adult (age, 81 years with end-stage idiopathic myelofibrosis). Two individuals (both with polycythemia rubra vera) presented with symptomatic intracranial or spinal canal lesions (age, 67 and 68 years). Mixed populations of immature hematopoietic cells were identified by characteristic morphology and appropriate immunohistochemistry. The precise pathogenesis of extramedullary hematopoiesis in central nervous system is unknown. In infants, extramedullary hematopoiesis is unlikely to be pathologic and most likely represents a resolving fetal state. The older children and adults with incidental or symptomatic extramedullary hematopoiesis usually have some underlying hematologic condition, which should be sought if not already recognized. (C) 2011 Elsevier Inc. All rights reserved.