Hematology/oncology clinics of North America
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Sickle cell disease (SCD) is the most commonly inherited hemoglobinopathy in the United States. Blood transfusion is a critical part of the multidisciplinary approach necessary in the management of SCD; however, blood transfusions are not without complications. The successful use of transfusion as a treatment strategy in SCD requires the critical review and knowledge of transfusion methods, generally accepted indications, clinical situations in which transfusion generally is not considered, the selection of blood products, and strategies to prevent transfusion-related complications.
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Hematol. Oncol. Clin. North Am. · Oct 2005
ReviewPulmonary hypertension in sickle cell disease: mechanisms, diagnosis, and management.
Pulmonary hypertension affects nearly one-third of adults with sickle cell disease and is related to hemolysis. Although pulmonary pressures are not as high as in other forms of pulmonary hypertension, sickle cell patients poorly tolerate even moderate elevations of pulmonary pressures, because this complication predicts short survival. ⋯ Because patients with pulmonary hypertension are older and often severely affected by other vaso-occlusive complications, optimizing their treatment with hydroxyurea or transfusions is important. Newer agents such as sildenafil and bosentan have demonstrated efficacy in other forms of pulmonary hypertension, and future clinical trials may prove them helpful in sickle cell patients.