Emerging treatments
Partial splenectomy
Due to the various risks associated with absence of splenic function, partial splenectomy has been explored. In one meta-analysis of 14 observational studies, full splenectomy was more effective than partial splenectomy at increasing haemoglobin levels and reducing reticulocytes at one year; approximately 5% of children who underwent partial splenectomy required completion splenectomy.[46] One subsequently published study reported no difference between total and partial splenectomy haemoglobin levels 5-years post-procedure.[47] A formal clinical trial and longer term follow-up is required to establish the place of this technique.[29][30][42]
Recombinant erythropoietin
Administration of erythropoietin has been shown to be potentially useful in reducing or eliminating the need for transfusions in neonates and infants with HS.[48][49] In one series, 13 of 16 infants with HS treated with recombinant erythropoietin were able to maintain adequate haemoglobin values to avoid transfusion.[48] Recombinant erythropoietin may be particularly useful in patients where transfusions are difficult, or in patients who prefer to avoid red blood cell transfusions (e.g., Jehovah's Witnesses).
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