Acquired hemoglobin H disease in idiopathic myelofibrosis.
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.
D000368:Aged; D005914:Globins / Q000096:biosynthesis; D006447:Hemoglobin H; D006455:Hemoglobins, Abnormal; D006801:Humans; D008297:Male; D055728:Primary Myelofibrosis / Q000097:blood* / Q000150:complications; D012333:RNA, Messenger / Q000378:metabolism; D012156:Reticulocytes / Q000378:metabolism; D013789:Thalassemia / Q000097:blood* / Q000150:complications
D006455:Hemoglobins, Abnormal; D012333:RNA, Messenger; D005914:Globins; D006447:Hemoglobin H