Thalassemia

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Related to Beta thalassemia: Alpha Thalassemia

thalassemia

[‚thal·ə′sē·mē·ə]
(medicine)
A hereditary form of hemolytic anemia resulting from a defective synthesis of hemoglobin: thalassemia major is the homozygous state accompanied by clinical illness, and thalassemia minor is the heterozygous state and may not have evident clinical manifestations. Also known as Mediterranean anemia.

Thalassemia

 

(also thalassanemia), a familial hemolytic anemia, first detected in 1925 among inhabitants of the Mediterranean region. Thalassemia is caused by disturbances in hemoglobin synthesis.

References in periodicals archive ?
Severe beta thalassemia is characterized by transfusion dependent anaemia, usually manifest in the first year of life (6-18 Month) when HbF declines normally.
The changing profile of homozygous beta thalassemia: demography, ethnicity, and age distribution of current North American patients and changes in two decades.
Conclusion: The overall frequency of beta thalassemia trait in our study was found to be 4.25%.
Due to technical constraints, this approach does not fully cure patients with severe forms of the disease, such as the one caused by the most common for the Cypriot population mutation of beta thalassemia," the statement said
When laboratory features are discordant for a diagnosis of beta thalassemia trait, it is useful to consider other conditions that affect the Hb [A.sub.2] level and red blood cell indices.
Frequency of hereditary hemochromatosis (HFE) gene mutations in Egyptian Beta thalassemia patients and its relation to iron overload.
Our study aimed at evaluating thyroid function in chronically transfused children with Beta Thalassemia Major in the first and second decade of life.
It should be noted that in some of the reported studies, comparing is performed between alpha-beta thalassemia with normal cases or in more than two groups but our emphasis was comparison between beta thalassemia carriers with alpha- beta thalassemia carriers in hematology indices so increasing amounts occur in very small scales (8,45).
Alpha and beta thalassemia. Am Fam Physician 2009; 80(4): 339-44.
Patients suffering from thalassemia and organ transplant are also considered at a high risk.5,6 Beta thalassemia major is a group of inherited recessive blood disorders characterized by abnormal formation of hemoglobin, known as hemoglobinopathies.The imbalance condition leads to red blood cells destruction in bone marrow which is followed by hemolysis and severe anemia.
Coexistent iron deficiency was seen in 51% of beta thalassemia patients.