Indications include

• Lymphoproliferative disorders, e.g. CLL, mantle zone lymphoma, hairy cell leukaemia. Reasons include massive organomegaly, occurrence of autoimmune complications and for diagnostic and/or therapeutic purposes.

• Myeloproliferative disorders —commonly used in myelofibrosis to reduce transfusion requirements, abdominal discomfort from massive splenic enlargement and may reduce constitutional symptoms e.g. weight loss and night sweats. Occasionally used in the management of chronic myeloid leukaemia.

• Autoimmune conditions —an accepted treatment in autoimmune thrombocytopenic purpura and autoimmune haemolytic anaemia following the failure of immunosuppression with corticosteroids and immunoglobulin (in the case of thrombocytopenic purpura). The procedure is not curative but may result in prolonged remissions and certainly will have steroid-sparing effect.

• Hereditary disorders —reduces red cell sequestration and transfusion requirements in homozygous g-thalassaemia. Recurrent, severe hereditary spherocytosis. Rare indications include pyruvate kinase deficiency and type 1 Gaucher's disease. Other circumstances where splenectomy may help include Felty's syndrome.

• Staging splenectomy is no longer a routine procedure for non-Hodgkin's lymphoma or Hodgkin's disease.

582 The clinician has to balance the risks and benefits of the procedure in an individual patient bearing in mind the long-term risk of post-splenectomy sepsis as well as immediate surgical factors. There are now established consensus guidelines for carrying out splenectomy.

Pre-operatively the need for the procedure is agreed with the patient and surgical team. At least 2 weeks pre-operatively immunisation with pneumococcal and Haemophilus vaccine should be given. Meningococcal vaccine may be offered but this covers sub-types A and C only and does not give long-lasting immunity. Peri-operative thromboembolic risks should be considered (e.g. standard surgical risks and those posed by the rebound thrombocytosis after splenectomy). Low dose heparin may be appropriate peri-operatively followed by low dose aspirin (may require modification in thrombocytopenia or if platelet dysfunction). Before discharge, patients must be given a leaflet/card which they carry. Life-long prophylaxis with penicillin V 250mg bd recommended or erythromycin 250mg bd if the patient is allergic to penicillin. The patient and his/her family must be advised to report urgently profound systemic symptoms, most promptly to their nearest local A&E department.

Re-vaccination with pneumococcal vaccine every 5 years recommended. Asplenic patients travelling to malarial areas must be meticulous in taking anti-malarial prophylaxis (greater risk of severe illness from Plasmodium falciparum).

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