Veer A, Kosciolek B A, Bauman A W, Rowley P T
Am J Hematol. 1979;6(3):199-206. doi: 10.1002/ajh.2830060304.
A 68-year-old male, diagnosed 1 year previously as having myelofibrosis, developed hemolysis, red cell inclusions, and 37% Hb H. The alpha/beta globin synthetic ratio for circulating reticulocytes, determined by 3H-leucine incorporation and globin chain separation by carboxymethylcellulose chromatography in urea, was 0.049. When total RNA was purified from peripheral blood cells and translated in a wheat germ cell-free translation system, the alpha/beta ratio of the translation products was 0.26, indicating mRNA as a major cause of the globin synthetic imbalance. This study demonstrates that myelofibrosis is one setting in which acquired Hb H disease occurs; that the synthetic imbalance may be extreme; and that it can be associated with an imbalance in the activities of specific globin mRNAs.
一名68岁男性，1年前被诊断为骨髓纤维化，出现溶血、红细胞包涵体以及37%的血红蛋白H。通过3H-亮氨酸掺入法和在尿素中用羧甲基纤维素色谱法分离珠蛋白链来测定循环网织红细胞的α/β珠蛋白合成比率，结果为0.049。当从外周血细胞中纯化总RNA并在麦胚无细胞翻译系统中进行翻译时，翻译产物的α/β比率为0.26，表明mRNA是珠蛋白合成失衡的主要原因。本研究表明，骨髓纤维化是获得性血红蛋白H病发生的一种情况；合成失衡可能非常严重；并且它可能与特定珠蛋白mRNA活性的失衡有关。
