Abstract
BACKGROUND: Chronic RBC exchange (RCE) is an established therapeutic strategy used to prevent the development of serious complications in patients with sickle cell disease and beta thalassemia. A subset of these patients have an accelerated decline of transfused red cells, leading to suboptimal exchange transfusions since the preprocedure hematocrit (HCT) is too low to allow for isovolemic hemodilution. These patients often have concomitant splenomegaly. METHODS: In our institution, we had 3 patients who had rapid decline in HCT post-RCE and who underwent a splenectomy. We compared the pre- and postsplenectomy hemoglobin S and HCT values for two patients with sickle cell anemia and one with beta thalassemia, undergoing chronic RCE. RESULTS: We observed a significant increase in the preprocedure HCT, from a mean ± SD of 21.11 (±2.5) presplenectomy to 25.02 (±1.8) postsplenectomy (p < 0.0001). This was accompanied by a significant increase in the interval number of days between procedures, from 29.6 (±5.6) days to 34.8 (±7.2) days following splenectomy (p=0.0046). Comparing pre- and postsplenectomy HCT values to the threshold HCT value required for isovolemic hemodilution (HCT = 23%) revealed that splenectomy resulted in a highly significant increase (p < 0.0001) above the threshold. DISCUSSION: Our observations here suggest that in a subset of patients, splenomegaly may result in accelerated decline of transfused red cells which improves following splenectomy, resulting in improved clinical parameters and more efficient RCE.