Anemias hemolíticas congénitas

The congenital hemolytic anemias are a group of genetically-based diseases characterized by an early destruction of red cells, with similar clinical and analytical characteristics. The high prevalence of many of them is explained by their protective effect against malaria. They can be due to membrane anomalies, of which the most common in our environment is hereditary spherocytosis, enzime defects, among which glucose-6-phosphate dehydrogenase deficiency stands out for its high prevalence, or hemoglobin alterations, amog which sickle cell disease and thalassemias are the most important. Their diagnosis is based on family history, clinical history and laboratory parameters, althoug the definitive diagnosis usually needs specialized mtheods, like molecular techniques. They lack specific treatment although some of them, like herditay spherocytosis, may respond to splenectomy and the most severe cases may need hematopoietic stem cell transplantation.