ACQUIRED HEMOGLOBIN-H DISEASE IN IDIOPATHIC MYELOFIBROSIS

A 68‐year‐old white 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, indicting 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. Copyright © 1979 Wiley‐Liss, Inc., A Wiley Company