medigraphic.com
ISSN 1561-2996 (Electronic)
ISSN 0864-0289 (Print)

2012, Number 3

<< Back Next >>

Rev Cubana Hematol Inmunol Hemoter 2012; 28 (3)

Splenectomy in sickle cell disease

Gutiérrez-Díaz A, Ramón-Rodríguez LG, Arencibia-Núñez A, Serrano-Mirabal J, Delgado-Vargas T, Anoceto-Martínez A, Jaime-Fagundo JC, González-Otero A, Svarch E

Language: Spanish
References: 44
Page: 235-245
PDF size: 66.62 Kb.


ABSTRACT

Splenic sequestration crisis is one of the most frequent and serious manifestations of sickle cell disease in young children. The standard treatment is total splenectomy, which has the risk of fulminant sepsis with high mortality. Other complications of total splenectomy are the increased incidence of some of the manifestations of the disease, and since a few years ago it is associated with predisposition to vascular events. At the Institute of Hematology and Immunology partial splenectomy is performed since 1986 with excellent results, as it eliminates almost completely the recurrence of acute splenic sequestration without increasing the risk of infections. It has been demonstrated that the splenic remainder retains its function and no risk of vascular complications has been reported.


REFERENCES

  1. Rees DC, Williams TN, Gladwin MT. Sickle-cell disease. Lancet. 2011 Dec 11;376(9757):2018-31.

  2. Ballas SK, Lieff S, Benjamin LJ, Dampier CD, Heeney MM, Hoppe C, et al. Definitions of the phenotypic manifestations of sickle cell disease. Am J Hematol. 2010 Jan;85(1):6-13.

  3. Brigden ML. Detection, education and management of the asplenic or hyposplenic patient. Am Fam Physician. 2001 Feb 1;63(3):499-506,508.

  4. Di Sabatino A, Carsetti R, Corazza GR. Post-splenectomy and hyposplenic states. Lancet. 2011 Jul 2;378(9785):86-97.

  5. Tomlinson WJ. Abdominal crises in sickle cell anemia: a clinicopathological study of eleven cases with a suggested explanation of their cause. Am J Med Sci. 1945;209:722-41.

  6. Al-Salem AH. Splenic Complications of sickle cell anemia and the role of splenectomy. ISRN Hematology [revista en la Internet]. 2011 [citado 2011 Sep 10]; 2011: 7 páginas. Disponible en: http://www.isrn.com/journals /hematology/2011/864257/cta/

  7. Svarch E, Marcheco B, Machín S, Menéndez A, Nordet I, Arencibia A, et al. La drepanocitosis en Cuba. Estudio en niños. Rev Cubana Hematol Inmunol Hemoter [serie en Internet]. 2011 Mar [citado 2011 Sep 19]; 27(1) Disponible en: http://scielo.sld.cu/scielo.php?script=sci_ arttext&pid=S0864-028920 11000100005&lng=es

  8. Kalpatthi R, Kane ID, Shatat IF, Rackoff B, Disco D, Jackson SM. Clinical events after surgical splenectomy in children with sickle cell anemia. Pediatr Surg Int. 2010 May;26(5):495-500.

  9. Svarch E, Vilorio P, Nordet I, Chesney A, Batista JF, Torres L, et al. Partial splenectomy in children with sickle cell disease and repeated episodes of splenic sequestration. Hemoglobin. 1996 Nov;20(4):393-400.

  10. Svarch E, Nordet I, Valdés J, Gonzalez A, Machín S, de la Torre E. Partial splenectomy in children with sickle cell disease. Haematologica. 2003 Feb;88(2):222-3.

  11. Harrod VL, Howard TA, Zimmerman SA, Dertinger SD, Ware RE. Quantitative analysis of Howell-Jolly bodies in children with sickle cell disease. Exp Hematol. 2007 Feb;35(2):179-83.

  12. Nouri A, de Montalembert M, Revillon Y, Girot R. Partial splenectomy in sickle cell syndromes. Arch Dis Child. 1991 Sep;66(9):1070-2.

  13. Pearson HA, McIntosh S, Ritchey AK, Lobel JS, Rooks Y, Johnston D. Developmental aspects of splenic function in sickle cell diseases. Blood. 1979 Mar;53(3):358-65.

  14. Wethers DL, Grover R. Reversibility of splenic function by transfusion in two young adults with sickle cell anemia. Am J Pediatr Hematol Oncol. 1987;9(3):209-11.

  15. Santos A, Pinheiro V, Anjos C, Brandalise S, Fahel F, Lima M, et al. Scintigraphic follow-up of the effects of therapy with hydroxyurea on splenic function in patients with sickle cell disease. Eur J Nucl Med Mol Imaging. 2002 Apr;29(4):536-41.

  16. Olivieri NF, Vichinsky EP. Hydroxyurea in children with sickle cell disease: impact on splenic function and compliance with therapy. J Pediatr Hematol Oncol. 1998 Jan-Feb;20(1):26-31.

  17. Sheikha AK, Salih ZT, Kasnazan KH, Khoshnaw MK, Al-Maliki T, Al-Azraqi TA, et al. Prevention of overwhelming postsplenectomy infection in thalassemia patients by partial rather than total splenectomy. Can J Surg. 2007 Oct;50(5):382-6.

  18. Timens W, Leemans R. Splenic autotransplantation and the immune system. Adequate testing required for evaluation of effect. Ann Surg. 1992 Mar;215(3):256-60.

  19. Vick LR, Gosche JR, Islam S. Partial splenectomy prevents splenic sequestration crises in sickle cell disease. J Pediatr Surg. 2009 Nov;44(11):2088-91.

  20. Svarch E, Nordet I, Gonzalez A. Overwhelming septicaemia in a patient with sickle cell/beta(0) thalassaemia and partial splenectomy. Br J Haematol. 1999 Mar;104(4):930.

  21. Bisharat N, Omari H, Lavi I, Raz R. Risk of infection and death among post-splenectomy patients. J Infect. 2001 Oct;43(3):182-6.

  22. Chatopadhaya B. Splenectomy, pneumococcal vaccination and antibiotic prophylaxis. Br J Hosp Med. 1989;41:1724.

  23. Barens JN, Dehodar HA, Marshall RT. Long term management after splenectomy. Br Med J. 1994;308:38.

  24. Working party of the British Committee for Standards in hematology, clinical hematology task force. Guidelines for prevention and treatment of infection in post splenectomy patients or those with dysfunctional spleen. Br Med J. 1996;312:430-4.

  25. Castro O, Brambilla DJ, Thorington B, Reindorf CA, Scott RB, Gillette P, et al. The acute chest syndrome in sickle cell disease: incidence and risk factors. The Cooperative Study of Sickle Cell Disease. Blood. 1994 Jul 15;84(2):643-9.

  26. Wales PW, Carver E, Crawford MW, Kim PC. Acute chest syndrome after abdominal surgery in children with sickle cell disease: Is a laparoscopic approach better? J Pediatr Surg. 2001 May;36(5):718-21.

  27. Haricharan RN, Roberts JM, Morgan TL, Aprahamian CJ, Hardin WD, Hilliard LM, et al. Splenectomy reduces packed red cell transfusion requirement in children with sickle cell disease. J Pediatr Surg. 2008 Jun;43(6):1052-6.

  28. Ghantous S, Al Mulhim S, Al Faris N, Abushullaih B, Shalak F, Yazbeck S. Acute chest syndrome after splenectomy in children with sickle cell disease. J Pediatr Surg. 2008 May;43(5):861-4.

  29. Piccin A, Smith OP, Murphy C, O'Marcaigh A, Corbally M, Mc Mahon C. Splenectomy in sickle cell anaemia: a cause of further crises? Br J Haematol. 2009 Apr;145(1):144-6.

  30. Goers T, Panepinto J, Debaun M, Blinder M, Foglia R, Oldham KT, et al. Laparoscopic versus open abdominal surgery in children with sickle cell disease is associated with a shorter hospital stay. Pediatr Blood Cancer. 2008 Mar;50(3):603-6.

  31. Hery G, Becmeur F, Mefat L, Kalfa D, Lutz P, Lutz L, et al. Laparoscopic partial splenectomy: indications and results of a multicenter retrospective study. Surg Endosc. 2008 Jan;22(1):45-9.

  32. Dutta S, Price VE, Blanchette V, Langer JC. A laparoscopic approach to partial splenectomy for children with hereditary spherocytosis. Surg Endosc. 2006 Nov;20(11):1719-24.

  33. Mohren M, Markmann I, Dworschak U, Franke A, Maas C, Mewes S, et al. Thromboembolic complications after splenectomy for hematologic diseases. Am J Hematol. 2004 Jun;76(2):143-7.

  34. Crary SE, Buchanan GR. Vascular complications after splenectomy for hematologic disorders. Blood. 2009 Oct 1;114(14):2861-8.

  35. Ataga KI. Hypercoagulability and thrombotic complications in hemolytic anemias. Haematologica. 2009 Nov;94(11):1481-4.

  36. Crary SE, Troendle S, Ahmad N, Buchanan GR. Traditional laboratory measures of cardiovascular risk in hereditary spherocytosis. Pediatr Blood Cancer. 2010 Oct;55(4):684-9.

  37. Ataga KI, Cappellini MD, Rachmilewitz EA. Beta-thalassaemia and sickle cell anaemia as paradigms of hypercoagulability. Br J Haematol. 2007 Oct;139(1):3-13.

  38. De Franceschi L, Cappellini MD, Olivieri O. Thrombosis and sickle cell disease. Semin Thromb Hemost. 2011 Apr;37(3):226-36.

  39. Van Beers EJ, Schaap MC, Berckmans RJ, Nieuwland R, Sturk A, van Doormaal FF, et al. Circulating erythrocyte-derived microparticles are associated with coagulation activation in sickle cell disease. Haematologica. 2009 Nov;94(11):1513-9.

  40. Cappellini MD, Robbiolo L, Bottasso BM, Coppola R, Fiorelli G, Mannucci AP. Venous thromboembolism and hypercoagulability in splenectomized patients with thalassaemia intermedia. Br J Haematol. 2000 Nov;111(2):467-73.

  41. Taher A, Isma'eel H, Mehio G, Bignamini D, Kattamis A, Rachmilewitz EA, et al. Prevalence of thromboembolic events among 8,860 patients with thalassaemia major and intermedia in the Mediterranean area and Iran. Thromb Haemost. 2006 Oct;96(4):488-91.

  42. Ataga KI, Moore CG, Hillery CA, Jones S, Whinna HC, Strayhorn D, et al. Coagulation activation and inflammation in sickle cell disease-associated pulmonary hypertension. Haematologica. 2008 Jan;93(1):20-6.

  43. van Beers EJ, Spronk HM, Ten Cate H, Duits AJ, Brandjes DP, van Esser JW, et al. No association of the hypercoagulable state with sickle cell disease related pulmonary hypertension. Haematologica. 2008 May;93(5):e42-4.

  44. Morris CR. Mechanisms of vasculopathy in sickle cell disease and thalassemia. Hematology Am Soc Hematol Educ Program. 2008:177-85.




2020     |     www.medigraphic.com