Indications for RBC Exchange Transfusion in Patients with Sickle Cell Disease: Revisited

Clinically, sickle cell disease (SCD) is characterized by chronic hemolytic anemia, recurrent acute vaso-occlusive crises, and progressive multi-organ failure. The management for SCD varies greatly among patients, and in the same patient longitudinally. Some patients may benefit from minimal therapy and infrequent transfusions, while others are transfusion dependent. Transfusion therapy can consist of simple transfusions, which often lead to iron overload and require iron chelation therapy. Simple transfusion, however, cannot reduce HbS levels rapidly, which may be required in certain critical conditions such as acute chest syndrome (ACS) or stroke. On the other hand, red blood cell exchange transfusion (RBCEX) can prevent iron overload and achieve rapid HbS reduction. In RBCEX, the red blood cells (RBCs) of the patient are replaced with normal RBCs, and the removed plasma is returned to the patient with minimal alteration. Plasma of patients with SCD is known to contain increased levels of several inflammatory mediators in the steady state that increase during crises. This finding explains why some patients with severe complications do not respond to RBCEX, but do much better after using plasma exchange in addition to RBCEX, as previously reported. Based on this, a revision of the indications for RBCEX in patents with SCD is recommended. DISCUSSION: In the 1980s and 1990s we used the Haemonetics Corporation's V-50 machine to perform intermittent whole blood (WB) exchange to treat severe complications of sickle cell disease (SCD.) The results were excellent, as previously described [1].