Recommendations regarding splenectomy in hereditary hemolytic anemias

Abstract

Hereditary hemolytic anemias are a group of disorders with a variety of causes, including red cell membrane defects, red blood cell enzyme disorders, congenital dyserythropoietic anemias, thalassemia syndromes and hemoglobinopathies. As damaged red blood cells passing through the red pulp of the spleen are 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 Roadmap we generated specific recommendations for each disorder, except thalassemia syndromes for which there are other, recent guidelines. Our recommendations are intended to enable clinicians to achieve better informed decisions on disease management by splenectomy, on the type of splenectomy and the possible consequences. As no randomized clinical trials, case control or cohort studies regarding splenectomy in these disorders were found in the literature, recommendations for each disease were based on expert opinion and were subsequently critically revised and modified by the Splenectomy in Rare Anemias Study Group, which includes hematologists caring for both adults and children.

Splenectomy indications and postoperative follow-up results of a department of general surgery

“…Splenectomy is currently being performed due to many benign and malign diseases such as erythrocyte disorders that cause platelet and erythrocyte dysfunction, hemoglobinopathies as well as cysts, abscesses, tumors, and ruptures of the spleen (12)(13)(14). Splenic abscess, splenic infarction, thalassemia, idiopathic thrombocytopenic purpura, agnogenic myeloid metaplasia, sarcoidosis, amyloidosis, Gaucher's and Niemann-Pick diseases, Felty syndrome are some of the splenectomy indications (13,(15)(16)(17)(18)(19)(20).…”

“…Splenic abscess, splenic infarction, thalassemia, idiopathic thrombocytopenic purpura, agnogenic myeloid metaplasia, sarcoidosis, amyloidosis, Gaucher's and Niemann-Pick diseases, Felty syndrome are some of the splenectomy indications (13,(15)(16)(17)(18)(19)(20). In addition to these, malign diseases of the spleen, invasion of malignant diseases and traumatic injuries are other indications (21)(22)(23).…”

Addressing the diagnostic gaps in pyruvate kinase deficiency: Consensus recommendations on the diagnosis of pyruvate kinase deficiency

“…Early onset of anemia is usually associated with a more severe clinical course. 4,42,44,45 Notably, since splenectomy is not indicated in some other forms of chronic hemolytic anemia, such as hereditary stomatocytosis, 46 the diagnosis of PK deficiency should be established and comorbidity of stomatocytosis or other thrombophilic disorders should be excluded before splenectomy is performed. The anemia tends to improve with age, and is relatively constant in adulthood, although exacerbations requiring occasional transfusion may occur because of the stress of acute infections or pregnancy.…”

Thrombocytosis and portal vein thrombosis after splenectomy for paediatric haemolytic disorders: How should they be managed?

“…Splenectomy is an effective intervention for selected children with haemolytic anaemia . Post‐splenectomy thrombocytosis (greater than 500 × 10 9 /L) occurs in at least 80% of patients, with platelet counts typically peaking between 1 and 3 weeks postoperatively .…”