Splenectomy in children with haematological disease.

During the years 1969-1980 elective splenectomy for haematological disease was performed in 31 children, of which 17 had haemolytic anaemia (HA), 11 thrombocytopenic purpura (TP) and 3 hypersplenism (HS). The indications for splenectomy in the HA series were haemolytic crises in 6 children, constant anaemia in 4, hyperbilirubinaemia in 5 and abdominal pain in 2 children. Among children with TP there were 8 cases of idiopathic thrombocytopenic purpura, 2 cases combined TP with haemolytic anaemia and idiopathic pancytopenia in 1 child. In all patients the indication for splenectomy was therapy-resistant thrombocytopenia. In the HS series the underlying disease was Morbus Gaucher, thyrosinosis and cysta lienalis. Indication for splenectomy was thrombocytopenia in all children. In the HA series the results of splenectomy were good in all patients. The haemoglobin value rose. The increased reticulocyte count returned to normal values. Splenectomy was considered to be still indicated in all patients with hereditary spherocytosis. In the TP series good results were obtained in 6 children, 2 were markedly improved, but 3 showed no change. Splenectomy plays an important role in the management of chronic thrombocytopenia when medical treatment is without effect. In the HS series thrombocytes reached normal values in all patients. Failure of splenectomy to relieve the haematologic process is usually due to remaining accessory spleens. Splenectomy in children increases the risk of severe infections and the polyvalent pneumococcal vaccine should therefore be administered prior to splenectomy.