Thalassemia Hemoglobin Research Articles

Dysesthesia of the mandible

A 33-year-old man with a history of sickle cell β-thalassemia (a combination of sickle hemoglobin [HbS] and thalassemia hemoglobin that has a diminished capacity of β-chain synthesis) was referred from the emergency department to the oral medicine clinic at Brigham and Women's Hospital, Boston, for management of acute onset of increasingly severe sharp pain throughout the right mandible. The patient reported having intense sharp shooting pain of the right mandible extending to the right temporomandibular joint, ear and forehead for three days.

Soluble and stroma-bound globin chains in mild and severe beta thalassemia.

In mild beta thalassemia hemoglobin levels are much higher than in severe beta thalassemia, yet black patients with the mild syndrome are as deficient in beta chain synthesis as severe thalassemia patients. The following studies were undertaken to determine if differences in the precipitation of excess alpha chain distinguishes these two groups of beta thalassemia homozygotes. The percentages of radioactive globin chain bound to erythroid cell stroma were determined in mild and severe thalassemia patients. Only the cells of splenectomized severe patients contained large amounts of stroma-bound globin radioactivity, the percentages in five such patients ranging from 6.7 to 13.9 (mean = 9.9). In four splenectomized patients with mild thalassemia the percentages ranged from 1.0 to 3.5 (mean = 1.9; p less than 0.005). This is the first observed difference in globin chain metabolism between these two groups of patients.

Inhomogeneities in Alkali-Resistant Hemoglobin. Demonstration of Zone Electrophoretic Differences Using a Cationic Detergent Electrolyte

Abstract Trimethyloctadecyl ammonium chloride, a cationic active detergent electrolyte, when added to starch gels before electrophoresis, caused selected alteration in the migration rates of alkali-resistant hemoglobin. Other red blood cell pigments were not similarly affected. Electrophoretic inhomogeneity of cord blood and thalassemia hemoglobin was noted. New components of cord blood hemoglobin were revealed by the method. A new component in the hemoglobin of cases of severe thalassemia was demonstrated.

Thalassemia–Hemoglobin C Disease: A New Syndrome Presumably due to the Combination of the Genes for Thalassemia and Hemoglobin C

Abstract An unusual type of severe chronic hypochromic microcytic anemia in a Negro is described and attributed to the interaction of the hemoglobin C gene with the thalassemia gene. The patient and his father were shown to be carriers of the C trait, the mother and several siblings of the thalassemia gene.