Abstract

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. The registry enrolled 355 pediatric patients (187 with sickle-cell disease/94 with thalassemia). Serum ferritin was the most frequent method used to detect iron overload. Complications from transfusional hemosiderosis were reported in ~80% of patients; hepatic (65·3%), endocrine (27·5%), and cardiac (18·2%) being the most frequent. These data indicate that hemoglobinopathies and complications due to transfusional hemosiderosis are a significant clinical problem in the Latin American population with iron overload. Chelation therapy is used insufficiently and has a high rate of discontinuation.

Links

Publisher Full Text

Authors

Lobo C, Angulo IL, Aparicio LR, Drelichman GI, Zanichelli MA, Cancado R, RELATH Investigators

Institution

Instituo Estadual de Hematologia Arthur de Siqueira Cavalcanti--HEMORIO, Rua Frei Caneca 8, Rio de Janeiro, Brazil. diretorio@hemorio.rj.gov.br

Source

Hematology (Amsterdam, Netherlands) 16:5 2011 Sep pg 265-73

MeSH

Adolescent
Adult
Aged
Aged, 80 and over
Blood Transfusion
Chelation Therapy
Child
Child, Preschool
Endocrine System Diseases
Female
Ferritins
Heart Diseases
Hemoglobins
Hemosiderosis
Humans
Iron Chelating Agents
Latin America
Liver Diseases
Male
Middle Aged
Registries
Retrospective Studies
Skin Pigmentation
Young Adult

Pub Type(s)

Journal Article

Language

eng

PubMed ID

21902889