Extramedullary hematopoiesis in the absence of myeloproliferative neoplasm: Mayo Clinic case series of 309 patients.
Extramedullary hematopoiesis in the absence of myeloproliferative neoplasm: Mayo Clinic case series of 309 patients.
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DOI：
10.1038/s41408-018-0156-6
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发表时间：
2018-11-19
影响因子：
12.8
通讯作者：
Tefferi A
中科院分区：
文献类型：
--
作者：
Fan N;Lavu S;Hanson CA;Tefferi A
髓外造血（EMH）意味着在骨髓外产生红系和髓系祖细胞。成人EMH通常见于骨髓增生性肿瘤（MPN）患者，但其与其他疾病（包括地中海贫血）的相关性早已被认识1。在MPN和非MPN环境中，肝脏和脾脏是EMH的两个最常见部位，并且已经假设循环造血细胞过滤可能通过内皮细胞表达的配体，如趋化因子配体12，而不是脾基质的包埋，解释了特定的现象2-4;在受影响患者的骨髓和脾组织中一致检测到MPN特异性突变，如JAK 2 V617 F，和特异性细胞遗传学异常，支持这一论点5，6.由于其他非特异性造血应激物而动员的循环造血祖细胞也涉及接种非肝脾EMH（NHS-EMH）7。后者已在多种器官中报道，包括中枢神经系统8、卵巢和输卵管9、皮肤10、肺和胸膜11、心包12、13、淋巴结14和其他部位。在之前发表的一份报告中，1975年至2002年15年间，马约诊所诊断的27例NHS-EMH病例中，最常见的相关疾病是骨髓纤维化，最常见的受累部位是胸椎。目前的研究重点是马约诊所的经验与EMH引用的MPN的情况下。目标是系统地
Extramedullary hematopoiesis (EMH) implies the production of erythroid and myeloid progenitor cells outside of the bone marrow. EMH in adults is typically seen in patients with myeloproliferative neoplasms (MPNs) but its association also with other conditions, including thalassemia, has long been recognized 1. In both MPN and non-MPN settings, the liver and spleen are the two most frequent sites of EMH and it has been hypothesized that circulating hematopoietic cell filtration (entrapment), possibly via endothelial cell expressed ligands, such as chemokine ligand 12, rather than splenic stroma account for the particular phenomenon 2–4; the concordant detection of MPN-specific mutations, such as JAK2V617F, and specific cytogenetic abnormalities in both bone marrow and splenic tissue of affected patients, supports this contention 5, 6.Circulating hematopoietic progenitors mobilized as a result of otherwise nonspecific hematopoietic stressors have also been implicated in seeding non-hepatosplenic EMH (NHS-EMH) 7. The latter has been reported in a variety of organs, including the central nervous system 8, ovaries and tubes 9, the skin 10, the lungs and pleura 11, the pericardium 12, 13, lymph nodes 14, and other sites. In a previously published report of 27 Mayo Clinic cases of NHS-EMH diagnosed antemortem between 1975 and 2002 15, the most common associated condition was myelofibrosis and the most frequent involved site the thoracic vertebral column. The current study focuses on the Mayo Clinic experience with EMH cited in the absence of MPN. The objectives were to systematically
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影响因子：
15.9
作者：
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