Hemoglobin SC disease and alpha-thalassemia. Prolonged survival and mild clinical course.
An 86-year-old black woman admitted for an elective cataract extraction was found to have moderate hypochromic microcytic anemia. Hematologic evaluation disclosed the presence of hemoglobin SC disease and heterozygous alpha-thalassemia-2 (alpha alpha/alpha-). A red cell density profile of the patient's peripheral blood revealed an absence of the typical uniform shift toward higher-density values seen in hemoglobin SC disease, indicating a "normalization" in the distribution of intracellular hemoglobin types. It is suggested that the co-inheritance of hemoglobin SC disease and heterozygous alpha-thalassemia-2, probably by decreasing the tendency toward intracellular hemoglobin S polymerization, contributed to her prolonged survival and relatively mild clinical course.
D000368:Aged; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000175:diagnosis / Q000235:genetics; D002874:Chromosome Mapping; D005260:Female; D006801:Humans; D013789:Thalassemia / Q000150:complications* / Q000175:diagnosis / Q000235:genetics