The Unusual Pathobiology of Hemoglobin Constant Spring Red Blood Cells
نویسندگان
چکیده
منابع مشابه
The unusual pathobiology of hemoglobin constant spring red blood cells.
Hemoglobin Constant Spring (HbCS) is the most common nondeletional alpha-thalassemic mutation and is an important cause of HbH-like disease in Southeast Asia. HbCS variants have an almost normal mean cell volume (MCV) and the anemia is more severe when compared with other alpha-thalassemic variants. We explored the pathobiology of HbCS red blood cells (RBCs) because the underlying cause(s) of t...
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Abstract Introduction: Hemoglobin Constant Spring (Hb CS), a abnormal Hb characterized by elongated α-globin chain resulting from mutations of the termination codon in the α2 - globin gene , is the most common nondelitional α-thalassemic mutation and is an important cause of HbH like disease in Southeast Asia. Case Report: A 9- years-old female with immune hemolytic anemia and splenomegally...
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237-40. 4. Hunt DM, Higgs DR, Winichagoon P, Clegg JB, Weatherall DJ. Haemoglobin Cs Sp has an unstable α chain messenger RNA. Br J Haematol 1982; 51:40513. 5. Harteveld CL, Traeger-Synodinos J, Ragusa A, et al. Multicentric origin of Hb Constant Spring [α2 codon 142 TAA-CAA]. Br J Haematol 1998; 102:50. 6. Pootrakul P, Winichagoon P, Fucharoen S, Pravatmuang P, Piankijagum A, Wasi P. Homozygou...
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The elongated alpha-globin chains of hemoglobin Constant Spring (alpha cs chain of HbCS ) are produced in low amounts such that the alpha cs-gene acts as a form of alpha-thalassemia; yet in the homozygous state the pathophysiological effects of this mutant are more severe than in the corresponding conditions that result from alpha-globin gene deletions. In studies designed to examine this discr...
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ژورنال
عنوان ژورنال: Blood
سال: 1997
ISSN: 1528-0020,0006-4971
DOI: 10.1182/blood.v89.5.1762