Caso contributivo
Quando la sferocitosi incontra il chirurgo
Surgical treatment of hereditary spherocytosis
Chiara Bibalo
Scuola di Specializzazione in Pediatria, IRCCS Materno-Infantile “Burlo Garofolo”, Università di Trieste
Giugno 2015
Abstract
Summary Hereditary spherocytosis is a common inherited disorder
that is characterized by anaemia, jaundice and splenomegaly.
It is reported worldwide and is the most common inherited
anaemia in individuals of northern European ancestry. Clinical
severity is variable with most of patients having a wellcompensated
haemolytic anaemia. Some individuals are
asymptomatic, whereas others have severe haemolytic anaemia
requiring erythrocyte transfusion. Common complications
are cholelithiasis and aplastic crises. Splenectomy is curative
and it is indicated in children with moderately-severe and severe
hereditary spherocytosis and in patients with symptoms of
cholelithiasis.
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Bibliografia
Bolton-Maggs PH, Langer JC, Iolascon A, Tittensor P, King MJ; General Haematology Task Force of the British Committee for Standards in Haematology. Guidelines for the diagnosis and management of hereditary spherocytosis--2011 update. Br J Haematol 2012;156(1):37-49.
del Giudice EM, Perrotta S, Nobili B, Specchia C, d'Urzo G, Iolascon A. Coinheritance of Gilbert syndrome increases the risk for developing gallstones in patients with hereditary spherocytosis. Blood 1999;94(7):2259-62.
Kliegman RM, Stanton BF, St. Geme J, Schor NF, Behrman RE. Pediatria. Nelson 18th edizione. Elsevier - Masson, 2012.
Perrotta S, Gallagher PG, Mohandas N. Hereditary spherocytosis. Lancet 2008;372 (9647):1411-26.
Roy SM, Buchanan GR, Crary SE. Splenectomy in children with "mild" hereditary spherocytosis. J Pediatr Hematol Oncol 2013;35(6):430-3.
Ruparel RK, Bogert JN, Moir CR, et al. Synchronous splenectomy during cholecystectomy for hereditary spherocytosis: Is it really necessary? J Pediatr Surg 2014;49:433-5.
Seims AD, Breckler FD, Hardacker KD, Rescorla FJ. Partial versus total splenectomy in children with hereditary spherocytosis. Surgery 2013;154(4):849-53.
Tamaro P, Barth E. Indicazioni alla splenectomia. Medico e Bambino 2000;19(6):373-5.
Tamary H, Aviner S, Freud E, et al. High incidence of early cholelithiasis detected by ultrasonography in children and young adults with hereditary spherocytosis. J Pediatr Hematol Oncol 2003;25(12):952-4.
Working Party of the British Committee for Standards in Haematology Clinical Haematology Task Force. Guidelines for the prevention and treatment of infection in patients with an absent or dysfunctional spleen. BMJ 1996;312(7028):430-4.
Wree A, Canbay A, Müller-Beissenhirtz H, et al. Excessive bilirubin elevation in a patient with hereditary spherocytosis and intrahepatic cholestasis. Z Gastroenterol 2011;49(8):977-80.
Yaish HM, Christensen RD, Agarwal A. A neonate with Coombs-negative hemolytic jaundice with spherocytes but normal erythrocyte indices: a rare case of autosomal-recessive hereditary spherocytosis due to alpha-spectrin deficiency. J Perinatol 2013;33(5):404-6.