Hb TAYBE: clinical and morphological findings IN 43 patients.

Unlabelled: Hereditary sequence variants in globin genes are usually silent and are rarer in α-globin chains than β-globin chains. Some may lead to an unstable protein with a hemolytic or thalassemic phenotype. Hb Taybe is an unstable α-chain hemoglobin variant caused by the deletion of a threonine residue at codon 38 or 39 of the α1 globin gene.

Prevention of β Thalassemia in Northern Israel - a Cost-Benefit Analysis.

Background: β Thalassemia major is characterized by hemolytic anemia, ineffective erythropoiesis and hemosiderosis. About 4% of the world population carries a Thalassemia gene. Management includes blood transfusions and iron chelation.

Sickle cell anemia in northern Israel: screening and prevention.

Background: Sickle cell anemia is a hemolytic anemia caused by a single mutation in position 6 of the beta globin molecule. About 80 patients with SCA in northern Israel are currently receiving treatment.

Objectives: To assess a screening program in northern Israel aimed at detecting couples at risk for having offspring with SCA.

Response to hydroxyurea therapy in beta-thalassemia.

Although a relatively small number of previous studies suggest a modest response to hydroxyurea (HU) therapy in beta-thalassemia, more recent investigations have revealed that some transfusion-dependent patients can become transfusion-independent following HU therapy. Patients with Ggamma XmnI polymorphism, several beta-globin mutations, and alpha-thalassemia deletions were inconsistently reported to have significant responses to HU therapy. To better predict who may respond, we retrospectively evaluated the clinical response and the molecular background of 18 beta-thalassemia patients treated with HU for a mean of 46 months.