Hemoglobin Gun Hill: An Unstable Protein Associated with Chronic Hemolysis

Hemoglobin Gun Hill: an unstable protein associated with chronic hemolysis.

Severe hemolytic anemia associated with the homozygous state for an unstable hemoglobin variant (Hb Bushwick).

We have investigated a 13-year-old girl from first cousin parents who presented with severe hemolytic anemia. Hematologic studies showed unstable hemoglobin (Hb) disease (chronic Heinz body anemia), and DNA analysis showed that the patient was homozygous for the previously reported abnormal Hb called Hb Bushwick (beta 74E18 gly-->val). Hb Bushwick is unstable in vitro and in vivo. In addition, ...

Elevated HbF associated with an unstable hemoglobin, hemoglobin Saint Etienne: Hb synthesis in blood BFUe in culture.

The red blood cells of a patient heterozygous for the unstable Hb Saint Etienne (p92 His -‘ GIn) contained 19% of fetal hemoglobin (Hb). Study of his family suggested that the proband had inherited the Swiss type of hereditary persistence of fetal hemoglobin (HPHF) from his father who had 1.2% of HbF and 8% of F cells. In order to gain further insight into the mechanism of the elevated producti...

Isozyme Associated with Premature Hemolysis

From the Departments of Medicine and Pathology, University of California Los Angeles School of Medicine, Los Angeles, California 90024, and the Veterans Administration Center, Los Angeles, California 90073; the Departments of Pediatrics and Internal Medicine, University of Rochester School of Medicine and Strong Memorial Hospital, Rochester, New York 14620; and the Department of Medicine, Dartm...

Oxidative Hemolysis and Precipitation of Hemoglobin

In a preceding report (1) it was shown that those redox compounds that cause Heinz body anemias in vivo produce a sequence of changes in vitro in solutions of crystalline hemoglobin culminating in the precipitation of hemoglobin as spherical granules. These granules appear to be identical with Heinz bodies. The sequence of changes produced in hemoglobin by these compounds includes: 1) the forma...