Agnogenic myeloid metaplasia: a clonal proliferation of hematopoietic stem cells with secondary myelofibrosis.
Agnogenic myeloid metaplasia: a clonal proliferation of hematopoietic stem cells with secondary myelofibrosis.
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不明原因的骨髓化生：造血干细胞的克隆性增殖，伴有继发性骨髓纤维化。
DOI：
10.1182/blood.v51.2.189.189
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发表时间：
1978
期刊：
影响因子：
20.3
通讯作者：
P. Fialkow
中科院分区：
文献类型：
--
作者：
Robert J. Jacobson;Armi Solo;P. Fialkow
葡萄糖-6-磷酸脱氢酶（G-6-PD）的类型和染色体的造血和其他组织与不可知性骨髓化生的妇女进行了测定。该患者在x连接的G-6-PD位点上是杂合的，因此在非造血细胞中发现了B和A同工酶。相比之下，在粒细胞、红细胞和血小板中只发现一种G-6-PD类型。她在血细胞中也有一种独特的染色体异常，但在其他组织中没有。这些结果表明，不可知性骨髓化生是一种多能干细胞的疾病，并提供了强有力的证据，证明它是克隆起源的。与血细胞相比，患者培养的骨髓“成纤维细胞”染色体正常，B型和A型G-6-PD均正常，提示骨髓纤维化是继发性异常。因此，至少在这个病例中，造血细胞增殖似乎是克隆的，由此推断，可能是肿瘤性的，而骨髓纤维化可能不是克隆的，因此似乎是继发性的。
The glucose-6-phosphate dehydrogenase (G-6-PD) types and chromosomes of hematopoietic and other tissues were determined in a woman with agnogenic myeloid metaplasia. The patient was heterozygous at the X-linked G-6-PD locus so that both B and A isoenzymes were found in nonhematopoietic cells. In contrast, only one G-6-PD type was found in granulocytes, red cells, and platelets. She also had a distinctive chromosome abnormality in blood cells but not in other tissues. These results indicate that agnogenic myeloid metaplasia is a disorder of a pluripotent stem cell and provide strong evidence that it is of clonal origin. In contrast to blood cells, the patient's cultured marrow "fibroblasts" had normal chromosomes and both B and A G-6-PD types, suggesting that the marrow fibrosis is a secondary abnormality. Thus, at least in this case of agnogenic myeloid metaplasia, the hematopoietic cell proliferation appears to be clonal, and, by inference, possibly neoplastic, whereas the marrow fibrosis is probably not clonal, and therefore appears to be secondary.