Recommendations regarding splenectomy in hereditary hemolytic anemias

Hereditary hemolytic anemias are a group of disorders including red cell membrane defects, red blood cells enzyme disorders, congenital dyserythropoietic anemias, thalassemia syndromes and haemoglobinopathies. As damaged red blood cells passing through the spleen red pulp are efficiently removed by splenic macrophages, splenectomy is one possible therapeutic approach to the management of severely affected patients. However, except for hereditary spherocytosis for which the effectiveness of splenectomy has been well documented, the efficacy of splenectomy in other anemias within this group has yet to be determined and there are concerns regarding short- and long-term infectious and thrombotic complications. In light of the priorities identified by the European Hematology Association (EHA) Roadmap we generated specific recommendations for each disorder that will enable clinicians to achieve better informed decisions on disease management by splenectomy, on the type of splenectomy and possible consequences. Guidelines for thalassemia syndromes that have been previously established were excluded from the current work. As no randomized clinical trials, case control or cohort studies regarding splenectomy in those disorders were found in the literature, recommendations for each disease were based on expert opinion, which were subsequently critically revised and modified by the Splenectomy in Rare Anemias Study Group including both adult and pediatric hematologists.