Alpha thalassemia and the hematology of homozygous sickle cell disease in childhood

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Alpha thalassemia and the hematology of homozygous sickle cell disease in childhood.

alpha Thalassemia modifies the hematologic expression of homozygous sickle cell (SS) disease, resulting in increased total hemoglobin and HbA2 and decreased HbF, mean cell volume, reticulocytes, irreversibly sickled cells, and bilirubin levels. The age at which these changes develop in children with SS disease is unknown. Ascertainment of globin gene status in a large representative sample of c...

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Thalassemia and the Hematology of Homozygous Sickle Cell Disease in Childhood

a Thalassemia modifies the hematologic expression of homozygous sickle cell (55) disease, resulting in increased total hemoglobin and HbA2 and decreased HbF. mean cell volume, reticulocytes. irreversibly sickled cells. and bilirubin levels. The age at which these changes develop in children with 55 disease is unknown. Ascertainment of globin gene status in a large representative sample of child...

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The First Observation of Homozygous Hemoglobin S-alpha Thalassemia Disease and Two Types of Sickle Cell Thalassemia Disease: (a) Sickle Cell-alpha Thalassemia Disease, (b) Sickle Cell-beta Thalassemia Disease.

S ICKLE CELL-THALASSEMIA DISEASE is known to be a severe or moderately severe type of congenital hemolytic anemia. The clinical and hematologic pictures of this hemoglobinopathy are usually similar to those found in the intermediate type of Cooley’s anemia; however, mild or asymptomatic cases have been reported by various investigators. 6 Singer et al.1 have described four Negro patients with m...

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The First Observation of Homozygous Hemoglobin S . Alpha Thalassemia Disease and Two Types of Sickle Cell

S ICKLE CELL-THALASSEMIA DISEASE is known to be a severe or moderately severe type of congenital hemolytic anemia. The clinical and hematologic pictures of this hemoglobinopathy are usually similar to those found in the intermediate type of Cooley’s anemia; however, mild or asymptomatic cases have been reported by various investigators. 6 Singer et al.1 have described four Negro patients with m...

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The First Observation of Homozygous Hemoglobin S.Alpha Thalassemia Disease and Two Types of Sickle Cell Thalassemia Disease : (a) Sickle Cell-Alpha Thalassemia Disease, (b) Sickle Cell-Beta Thalassemia Disease

S ICKLE CELL-THALASSEMIA DISEASE is known to be a severe or moderately severe type of congenital hemolytic anemia. The clinical and hematologic pictures of this hemoglobinopathy are usually similar to those found in the intermediate type of Cooley’s anemia; however, mild or asymptomatic cases have been reported by various investigators. 6 Singer et al.1 have described four Negro patients with m...

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ژورنال

عنوان ژورنال: Blood

سال: 1986

ISSN: 0006-4971,1528-0020

DOI: 10.1182/blood.v67.2.411.411