Polycythemia vera. Further in vitro studies of hematopoietic regulation.
Polycythemia vera. Further in vitro studies of hematopoietic regulation.
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真性红细胞增多症。
DOI：
10.1172/jci109989
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发表时间：
1980
期刊：
影响因子：
--
通讯作者：
Fialkow,PJ
中科院分区：
文献类型：
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作者：
Adamson,JW;Singer,JW;Catalano,P;Murphy,S;Lin,N;Steinmann,L;Ernst,C;Fialkow,PJ
进一步对2例真性红细胞增多症患者进行了体外造血调节研究，这些患者都是葡萄糖-6-磷酸脱氢酶(G-6-PD)杂合子(GDB/GDA)。在循环红细胞、粒细胞和血小板中仅检出G-6-PD A型，而在1例患者外周血和骨髓培养中发现大量G-6-PD B型红系祖细胞(BFU-E)和粒/巨噬细胞集落形成单位。详细的分析表明：(A)在可检测到的情况下，发现正常的BFU-E和粒/巨噬细胞集落形成单位具有相似的频率；(B)外周血和骨髓培养的正常祖细胞的频率相同；(C)BFU-E与更成熟的红系集落形成单位之间的正常红系分化受到抑制；(D)正常集落形成单位的患病率随着时间的推移而下降，这表明疾病的进展与异常克隆的产物进一步抑制正常造血有关。
Further in vitro studies of hematopoietic regulation were carried out in two patients with polycythemia vera who were also heterozygotes (GdB/GdA) for glucose-6-phosphate-dehydrogenase (G-6-PD). While only G-6-PD type A was detectable in circulating erythrocytes, granulocytes and platelets, cultures of peripheral blood and marrow from one patient revealed a substantial number of G-6-PD type B erythroid burst-forming units (BFU-E) and granulocyte/macrophage colony-forming units. Detailed analysis demonstrated: (a) where detectable, normal BFU-E and granulocyte/macrophage colony-forming units were found with similar frequencies; (b) the same frequencies for normal progenitors characterized cultures of peripheral blood and marrow; (c) inhibition of normal erythroid differentiation between BFU-E and the more mature erythroid colony-forming unit; (d) a decline in the prevalence of normal colony-forming units with time, suggesting that disease progression is associated with further suppression of normal hematopoiesis by products of the abnormal clone.