Regular long-term red blood cell transfusions for managing chronic chest complications in sickle cell disease

Abstract

Background-Sickle cell disease is a genetic haemoglobin disorder, which can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Sickle cell disease is one of the most common severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta globin) genes. The two most common chronic chest complications due to sickle cell disease are pulmonary hypertension and chronic sickle lung disease. These complications can lead to morbidity (such as reduced exercise tolerance) and increased mortality.

Red blood cell exchange: 2015 American Society for Apheresis consensus conference on the management of patients with sickle cell disease

“…In conclusion, all 3 studies which used right heart catheterization to diagnose PHT found that: 1) the prevalence of PHT in adult patients with SCD is 6‐10.5%, 2) PHT is a risk factor for death, and 3) PHT is associated with hemolytic anemia …”

“…Although patients with PHT are receiving chronic transfusion therapy, the data to support this practice is minimal. A recent Cochrane report aimed at evaluating chronic red cell transfusion for managing chronic chest complications in SCD, including PHT, found no relevant studies . A recent publication reports 2 patients with SCD complicated by PHT who were managed with chronic automated RBCx .…”

Red blood cell exchange: 2015 American Society for Apheresis consensus conference on the management of patients with sickle cell disease

“…In conclusion, all 3 studies which used right heart catheterization to diagnose PHT found that: 1) the prevalence of PHT in adult patients with SCD is 6‐10.5%, 2) PHT is a risk factor for death, and 3) PHT is associated with hemolytic anemia …”

“…Although patients with PHT are receiving chronic transfusion therapy, the data to support this practice is minimal. A recent Cochrane report aimed at evaluating chronic red cell transfusion for managing chronic chest complications in SCD, including PHT, found no relevant studies . A recent publication reports 2 patients with SCD complicated by PHT who were managed with chronic automated RBCx .…”

Guidelines on red cell transfusion in sickle cell disease Part II : indications for transfusion

“…Transfusion has also been used in patients undergoing liver transplantation in the peri‐transplant setting (Blinder et al , ; Gardner et al , ). Blood transfusion in the treatment of pulmonary hypertension seems theoretically reasonable, with the aim of decreasing haemolysis and thereby nitric oxide scavenging and consequent pulmonary vasoconstriction, and has been recommended for this purpose (Machado & Gladwin, ; Cho & Hambleton, ).…”

Regular automated red cell exchange transfusion in the management of pulmonary hypertension in sickle cell disease

“…Pulmonary hypertension (PHT) is a recognized complication of sickle cell disease (SCD) that is associated with significant morbidity and mortality (De Castro et al , ). Although the beneficial role of transfusion in SCD is well established, there is a surprising paucity of direct evidence on the use of transfusion and, especially, automated red cell exchange transfusion (ARCET) for the treatment of patients with PHT (Cho & Hambleton, ).…”

Red blood cell exchange: 2015 American Society for Apheresis consensus conference on the management of patients with sickle cell disease

“…In conclusion, all 3 studies which used right heart catheterization to diagnose PHT found that: 1) the prevalence of PHT in adult patients with SCD is 6‐10.5%, 2) PHT is a risk factor for death, and 3) PHT is associated with hemolytic anemia …”

“…Although patients with PHT are receiving chronic transfusion therapy, the data to support this practice is minimal. A recent Cochrane report aimed at evaluating chronic red cell transfusion for managing chronic chest complications in SCD, including PHT, found no relevant studies . A recent publication reports 2 patients with SCD complicated by PHT who were managed with chronic automated RBCx .…”

Guidelines on red cell transfusion in sickle cell disease Part II : indications for transfusion

“…Transfusion has also been used in patients undergoing liver transplantation in the peri‐transplant setting (Blinder et al , ; Gardner et al , ). Blood transfusion in the treatment of pulmonary hypertension seems theoretically reasonable, with the aim of decreasing haemolysis and thereby nitric oxide scavenging and consequent pulmonary vasoconstriction, and has been recommended for this purpose (Machado & Gladwin, ; Cho & Hambleton, ).…”

Regular automated red cell exchange transfusion in the management of pulmonary hypertension in sickle cell disease

“…Pulmonary hypertension (PHT) is a recognized complication of sickle cell disease (SCD) that is associated with significant morbidity and mortality (De Castro et al , ). Although the beneficial role of transfusion in SCD is well established, there is a surprising paucity of direct evidence on the use of transfusion and, especially, automated red cell exchange transfusion (ARCET) for the treatment of patients with PHT (Cho & Hambleton, ).…”

Red blood cell exchange: 2015 American Society for Apheresis consensus conference on the management of patients with sickle cell disease

“…In conclusion, all 3 studies which used right heart catheterization to diagnose PHT found that: 1) the prevalence of PHT in adult patients with SCD is 6‐10.5%, 2) PHT is a risk factor for death, and 3) PHT is associated with hemolytic anemia …”

“…Although patients with PHT are receiving chronic transfusion therapy, the data to support this practice is minimal. A recent Cochrane report aimed at evaluating chronic red cell transfusion for managing chronic chest complications in SCD, including PHT, found no relevant studies . A recent publication reports 2 patients with SCD complicated by PHT who were managed with chronic automated RBCx .…”

Guidelines on red cell transfusion in sickle cell disease Part II : indications for transfusion

“…Transfusion has also been used in patients undergoing liver transplantation in the peri‐transplant setting (Blinder et al , ; Gardner et al , ). Blood transfusion in the treatment of pulmonary hypertension seems theoretically reasonable, with the aim of decreasing haemolysis and thereby nitric oxide scavenging and consequent pulmonary vasoconstriction, and has been recommended for this purpose (Machado & Gladwin, ; Cho & Hambleton, ).…”

Regular automated red cell exchange transfusion in the management of pulmonary hypertension in sickle cell disease

“…Pulmonary hypertension (PHT) is a recognized complication of sickle cell disease (SCD) that is associated with significant morbidity and mortality (De Castro et al , ). Although the beneficial role of transfusion in SCD is well established, there is a surprising paucity of direct evidence on the use of transfusion and, especially, automated red cell exchange transfusion (ARCET) for the treatment of patients with PHT (Cho & Hambleton, ).…”