Clinical and hematological effects of hydroxyurea therapy in sickle cell patients: a single-center experience in Brazil.

CONTEXT AND OBJECTIVES Sickle cell disease (SCD) is the most common genetic disorder among people of African descent, affecting approximately 3,500 newborns each year in Brazil. Hydroxyurea (HU) is the only effective drug to treating patients with SCD, thereby reducing morbidity and mortality. The objective was to analyze the effects of HU on SCD patients at our institution.

Hematological differences between patients with different subtypes of sickle cell disease on hydroxyurea treatment.

Objective: Sickle cell anemia and the interaction S/Beta thalassemia differ in hematological values due to microcytosis and hypochromia caused by the thalassemic mutation. The clinical benefit of long-term hydroxyurea treatment is undeniable in sickle cell disease with monitoring of the biological action of the drug being by the complete blood count. The objective of this work is to compare changes in some of the erythrocytic indexes between S/Beta thalassemia and sickle cell anemia patients on long-term hydroxyurea treatment.

Retrospective epidemiological study of Latin American patients with transfusional hemosiderosis: the first Latin American epidemiological study in iron overload--the RELATH study.

The retrospective epidemiological study of Latin Americans with transfusional hemosiderosis is the first regional patient registry to gather data regarding the burden of transfusional hemosiderosis and patterns of care in these patients. Retrospective and cross-sectional data were collected on patients ≥2 years with selected chronic anemias and minimum 20 transfusions. In the 960 patients analyzed, sickle-cell disease (48·3%) and thalassemias (24·0%) were the most frequent underlying diagnoses.

Liver iron concentration evaluated by two magnetic methods: magnetic resonance imaging and magnetic susceptometry.

Quantification of liver iron concentration (LIC) is crucial in the management of patients suffering from certain pathologies that can produce iron overload, such as Cooley's anemia and hemochromatosis. All of these patients must control the level of iron deposits in their organs to avoid the toxicity of high LIC, which is potentially lethal. This paper describes experimental protocols for LIC measurement using two magnetic techniques: magnetic resonance imaging (MRI) and biomagnetic liver susceptometry (BLS).