Sickle cell syndromes. II. The sickle cell anemia-alpha-thalassemia syndrome |
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Authors: | G R Honig M Koshy R G Mason L N Vida |
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Affiliation: | 1. Children''s Memorial Hospital, Department of Pediatrics, Northwestern University, Chicago, Ill. USA;2. Michael Reese Medical Center, Division of Hematology, Department of Medicine, Chicago, Ill. USA |
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Abstract: | Five American black patients, ages 1 to 16 years, with the sickle cell anemia-alpha-thalassemia syndrome are described. Each patient had persistent microcytosis not explained by iron deficiency, and in each family the presence of alpha-thalassemia in combination with sickle cell trait was demonstrated in one of the parents. In one patient, in whom the diagnosis of sickle cell anemia was established at birth, an elevated level of Barts (gamma4) hemoglobin was also found. In these patients levels of alkali-resistant hemoglobin and reticulocyte counts were similar to those of sickle cell anemia patients of comparable age; however, stained smears of their peripheral blood rarely showed the presence of irreversibly sickled cells. No major ameliorative effect of the alpha-thalassemia on the clinical expression of the sickle cell disease of these patients was evident. |
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Keywords: | Reprint address: Division of Hematology Children's Memorial Hospital 2300 Children's Plaza Chicago IL 60614. |
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