Thalassemia disease is a common inherited hemolytic anemia frequently found in several parts of the world, especially in the Mediterranean and some Asian countries. Besides the complications of secondary hemochromatosis from regular red blood cell (RBC) transfusion and increased gastrointestinal absorption of iron, thromboembolism (TE) is one of the common long-term complications of beta-thalassemia disease, particularly in patients with non-transfusion-dependent thalassemia (NTDT), which is commonly seen after the second decade of life. The risk factors of TE in beta-thalassemia disease including exposure of phosphatidylserine of abnormal RBCs, increase of platelet activation and aggregation, elevation of endothelial microparticles and increased endothelial activation, decreased nitric oxide (NO) secondary to hemolysis, rise of platelet count and nucleated RBCs after splenectomy, organ dysfunction caused by hemochromatosis, and thrombophilia such as natural anticoagulant deficiencies leading to hypercoagulable state. The understanding of the pathophysiology would result in effective prevention of this complication of beta-thalassemia disease.
Part of the book: Beta Thalassemia
Bleeding and thrombosis are the common hematological complications found in children who are admitted in the pediatric intensive care units (PICUs). Some of those complications could be mild, however some could be serious or life-threatening for critically-ill children. The etiologies of those conditions could be due to the underlying diseases, i.e., congenital bleeding disorders, complications of the diseases, i.e. coagulopathy due to disseminated intravascular coagulation (DIC), and also the side effects from the treatments themselves, i.e., massive transfusion or extracorporeal membrane oxygenation (ECMO). Early detection and management and prevention of those complications could decrease the morbidity and mortality of the children in PICUs. Although most guidelines of management of those bleeding and thrombosis in adults is well established, the evidences for the management of those conditions in children are limited. In addition, developmental hemostasis during the childhood, which is different from adulthood, could challenge the management of those conditions in children admitted in PICUs.
Part of the book: ICU Management and Protocols