beta-thalassemia

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thalassemia

 [thal″ah-se´me-ah]
a heterogeneous group of hereditary hemolytic anemias marked by a decreased rate of synthesis of one or more hemoglobin polypeptide chains, classified according to the chain involved (α, β, δ); the two major categories are α- and β-thalassemia.
α-thalassemia (alpha-thalassemia) that caused by diminished synthesis of alpha chains of hemoglobin. The homozygous form is incompatible with life, the stillborn infant displaying severe hydrops fetalis. The heterozygous form may be asymptomatic or marked by mild anemia.
β-thalassemia (beta-thalassemia) that caused by diminished synthesis of beta chains of hemoglobin. The homozygous form is called t. major and the heterozygous form is called t. minor.
thalassemia ma´jor the homozygous form of β-thalassemia, in which hemoglobin A is completely absent; it appears in the newborn period and is marked by hemolytic, hypochromic, microcytic anemia; hepatosplenomegaly; skeletal deformation; mongoloid facies; and cardiac enlargement.
thalassemia mi´nor the heterozygous form of β-thalassemia; it is usually asymptomatic, but there may be mild anemia.
sickle cell–thalassemia a hereditary anemia involving simultaneous heterozygosity for hemoglobin S and thalassemia.

beta-thalassemia

an anemia that is caused by diminished synthesis of beta chains of hemoglobin. The homozygous form is known as thalassemia major and the heterozygous form is known as thalassemia minor. See thalassemia.

β-thalassemia

Thalassemia major Hematology A hemoglobinopathy caused by a defect in the synthesis of Hb β chain Clinical Pallor, fatigability, FTT, fever due to infections, diarrhea, bone deformities, hepatosplenomegaly Management Transfusions, but iron overload can damage the heart, liver, and endocrine systems, ergo iron chelation–early use of deferiprone, deferoxamine ↓ transfusion-related iron overload and may protect against DM, cardiac disease, early death

beta-thalassemia (bāˑ·t-tha·ls·sēˑ·mē·),

n type of anemia occurring as a result of reduced synthesis of the beta chains of hemoglobin.

be·ta-thal·as·se·mi·a

(bātă thală-sēmē-ă)
Thalassemia due to one of two or more genes that depress (partially or completely) synthesis of beta-globin chains by the chromosome bearing the abnormal gene.
References in periodicals archive ?
Beta-thalassemias are broadly classified into three groups, based on clinical severity: major, intermedia and minor.
To the best of our knowledge, the relationship between beta-thalassemia minor and NO has not been identified in the literature.
The objective of this study was to demonstrate plasma NO levels in beta-thalassemia minor patients.
The beta-thalassemias can be divided into several varieties.
Alpha-thalassemia depresses only the production of the alpha chains, and beta-thalassemia depresses only the production of the beta chains.
At Johns Hopkins University, David Weatherall and associates labeled reticulocytes of thalassemic patients with radioactive amino acids in vitro and were able to demonstrate that in patients with alpha- and beta-thalassemia, alpha- or beta-chain production was defective because of unbalanced globin chain synthesis (21).
Endocrine involvement in children with beta-thalassemia major.
Thyroid function in patients with homozygous beta-thalassemia.
Endocrine functioning in multitransfused prepubertal patients with homozygous beta-thalassemia.
a biopharmaceutical company developing protein therapeutics for cancer and orphan diseases, presented data today at the 3rd Pan-European Conference on Hemoglobinopathies and Rare Anemias in Limassol, Cyprus, demonstrating positive effects of ACE-536 to treat both the severe anemia and serious organ damage due to iron overload in a preclinical model of beta-thalassemia.
In a preclinical mouse model of beta-thalassemia intermedia, ACE-536 significantly increased levels of RBCs and hemoglobin by promoting the differentiation of late stage RBC precursors.
We are excited by these data which show the potential of ACE-536 to substantially alleviate the anemia and iron overload characteristic of the disease and look forward to initiating a Phase 2 trial of ACE-536 in patients with beta-thalassemia in the next few months.