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Acquired hemoglobin H disease in idiopathic myelofibrosis
Author(s) -
Veer Ashabala,
Kosciolek Barbara A.,
Bauman Arthur W.,
Rowley Peter T.
Publication year - 1979
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830060304
Subject(s) - myelofibrosis , globin , hemoglobin e , medicine , hemoglobin , hemolysis , rna , translation (biology) , microbiology and biotechnology , immunology , messenger rna , biology , biochemistry , gene , bone marrow
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 3 H‐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.