Thalassemia is a group of hereditary hemoglobinopathies. It is one of the most
common genetic disorders worldwide, presenting major public health and social
challenges in high incidence areas. Thalassemia is inherited in an autosomal
recessive manner. It is manifested as chronic hemolytic anemia, which is caused by
partial or complete lack of the synthesis of alpha- or beta-globulin chains that form
hemoglobin. Thalassemia major (TM) is associated with the most serious clinical
changes and life-threatening risk and is characterized by the triad of chronic
anemia, ineffective erythropoiesis, and iron overload. Anemia can be treated with
regular blood transfusions, but this life-saving therapy results in a “second disease”
due to iron accumulation in the body tissues. Iron overload is the main cause of
morbidity and mortality. The oral and maxillofacial features of TM are protruding
frontal and malar bones, thinning of the mandibular inferior cortex, small maxillary
sinuses, maxillary hypertrophy, and flaring of the maxillary anterior teeth. Dental
complications of TM include dental caries, periodontal disease, reduction in tooth
size, teeth spacing, short and narrow dental arches, delayed tooth development,
malocclusion. This monograph discusses the epidemiology, pathophysiology,
clinical manifestations, radiological characteristics, dental care, management and
complications. Guidelines for dental care are presented and stratiges of thalassemia
prevention are reviewed.
Keywords: Clinical features, Complications, Consanguinity, Epidemiology,
Management, Pathophysiology, Prevention, Thalassemia.
