Scientific Comments advised, the evidence for this is scanty.(4) It may be more important to advise patients carefully about the need for urgent medical attention for fever, and to keep a course of appropriate antibiotic at home to start when a suspected infection starts. These are not the only organisms with risk; the spleen gives some protection against malaria and this might affect the balance when considering whether to perform splenectomy in malaria areas. The high incidence of splenic sequestration reported in this survey (14 cases – 22.2%) is surprising. This is not a common complication of HS and is rarely reported in the literature and is not generally among the indications for splenectomy in this condition. Could these cases have been precipitated by intercurrent viral infections? In HS the indications for splenectomy must be carefully weighed up. There is increasing recognition of additional long term risks associated with thrombosis and vascular disease.(5) The clinical picture of HS may be modified by co-inheritance of haemoglobinopathies (sickle cell trait, thalassaemia) which show considerable geographical variations. These can affect the degree of anaemia and the risk of cholelithiasis, and need to be taken into consideration in the overall management strategy. Gall stones are a recognised complication of HS due to increased bilirubin turnover and the risk is reduced considerably by splenectomy. It is not clear whether regular ultrasonographic evaluations is cost-effective in the asymptomatic patient(3) and it is not clear whether asymptomatic stones should be treated surgically. The recent guidelines(3) recommend that, in children undergoing splenectomy, the gallbladder should be removed at the same time if there are symptomatic gallstones. If the gallbladder is left in situ, including cases when a cholecystostomy with stone extraction is done, close follow-up using ultrasound xxx is necessary. In children who require cholecystectomy for symptoms of gallstones, the use of concurrent splenectomy is controversial. It may be associated with a decreased future risk of common bile duct stones, but is associated with a risk of post-splenectomy sepsis. The laparoscopic route is recommended, but this depends upon local facilities and an experienced surgeon. It is important to make the correct diagnosis, to provide the patient and family with good information and t o e n c o u r a g e n o r m a l a c t i v i t y a n d l i f e s t y l e . C a r e f u l assessment should be made before proceeding to surgery and older affected relatives treated by splenectomy in the past may need advice and immunisations in relation to their risk of infection. References 1. Oliveira MC, Fernandes RA, Rodrigues CL, Ribeiro DA, Giovanardi MF, Viana MB. Clinical course of 63 children with hereditary spherocytosis: a retrospective study. Rev Bras Hematol Hemoter. 2012;34(1):9-13. 2. Eber SW, Armbrust R, Schroter W. Variable clinical severity of hereditary spherocytosis: relation to erythrocytic spectrin concentration, osmotic fragility and autohemolysis. J Pediatr. 1990;177(3):409-16. 3. Bolton-Maggs PH, Langer JC, Iolascon A, Tittensor P, King MJ. Guidelines for the diagnosis and management of hereditary spherocytosis - 2011 update. Br J Haematol. 2012;156(1):37-49. 4. Davies JM, Lewis MP, Wimperis J, Rafi I, Ladhani S, Bolton-Maggs PH. Review of guidelines for the prevention and treatment of infection in patients with an absent or dysfunctional spleen: prepared on behalf of the British Committee for Standards in Haematology by a working party of the Haemato-Oncology task force. Br J Haematol. 2011;155(3):308-17. 5. Crary SE, Buchanan GR. Vascular complications after splenectomy for hematologic disorders. Blood. 2009;114(14):2861-8. Comment in: Blood. 2009;114(26):5404. 4 Rev Bras Hematol Hemoter. 2012;34(1):3-8