SPLENECTOMY IN HEREDITARY HEMOLYTIC ANEMIA : 82 TUNISIAN CASES

##plugins.themes.academic_pro.article.sidebar##

Download
pdf
Vol. 87 No. 5 (2009): Issue May 2009

##plugins.themes.academic_pro.article.main##

Raouf Hafsia
Sami Zriba
Emna Gouider
Naouel Ben Salah
Wejdane Borji
Abdejelil Zaouche
Service de chirurgie Hôpital Charles Nicole - Tunis

Abstract

Background: Splenectomy is frequently advised in hereditary hemolytic anemia.Severe complications could occur after splenectomy.
Aim: To provide the indication and benefit of splenectomy
Methods: clinical and biological patterns were performed in a retrospective study of 82 patients: 17 homozygous beta thalassemia, 17 thalassemia intermedia, 33 heterozygote HbS / beta thalassemia and 15 hereditary spherocytosis.
Results: Splenectomy was performed for: Hypertransfusion in homozygous thalassemia, hereditary spherocytosis; hypersplenism in Thalassemia intermedia and splenic sequestration in heterozygote HbS / beta thalassemia.The benefit of splenectomy was proved in hereditary spherocytosis (100%), heterozygote HbS / beta
thalassemia (90%) and thalassemia intermedia (75%);nevertheless in homozygous beta thalassemia.Post splenectomical complication are often thrombocytosis, thrombosis and infections.
Conclusion:Splenectomy should be performed in hereditary hemolytic anemia to reduce and avoid transfusion.

Keywords:

hereditary hemolytic anemia, splenectomy, transfusion, complications

##plugins.themes.academic_pro.article.details##

References

  1. Weatherall DJ,Provan AB. Red cells I: inherited anaemias. The Lancet 2000; 355:1169-75.
  2. M de Montalembert. Transfusion des patients atteints d'hémoglobinopathies. Clin Biol 2000; 7:553-8.
  3. Bolton-Maggs PHB, Stevens RF, Dodd NJ, Lamont G, Tittensor P, King MJ on behalf of the General Haematology Task Force of the British Committee for Standards in Haematology. Guidelines for the diagnosis and management of hereditary spherocytosis. Br J Haematol 2004; 126:455-74.
  4. Rund D, Rachmilewitz E. ‚-Thalassemia. N Engl J Med 2005; 353:1135-46.
  5. Piomelli S. The management of patients with Cooley's anemia : transfusions and splenectomy. Semin Hematol 1995; 32:262-8.
  6. Taher A,Isma'eel H, Cappellini MD. Thalassemia intermedia: Revisited. Blood Cells Mol Dis 2006; 37:12-20.
  7. Al-Salem AH, Naserullah Z, Qaisaruddin S, Al-Abkari H, Al-Faraj A, Yassin YM. Splenic complications of the sickling syndromes and the role of splenectomy. J Pediatr Hematol Oncol 1999; 21:401-6.
  8. Weatherall DJ. ABC of clinical haematology: the hereditary anaemias. BMJ 1997; 314:492-6
  9. Bader-Meunier B, Gauthier F, Archambaud F, Cynober T, Miélot F, Dommergues JP, Warszawski J, Mohandas N, Tchernia G. Longterm evaluation of the beneficial effect of subtotal splenectomy for management of hereditary spherocytosis. Blood 2001; 97:399-403.
  10. Koffi KG, Sanogo I, Toure AH, Allangba OT, N'dri Oka D, Aguehounde C, Sangare A. Indications des splénectomies au cours des hémoglobinopathies majeures: à propos de 21 cas. Méd Afr Noire 2000; 47:426-9.
  11. Cario H, Stahnke K, Sander S, Kohne E. Epidemiological situation and treatment of patients with thalassemia major in Germany: results of the German multicenter ‚-thalassemia study.Ann Hematol 2000;79:7-12.
  12. Aessopos A, Farmakis D, Defteres S, Tsironi M, Polonifi A, Moyssakis I, Diamanti Kandaraki E, Papalambros E. Cardiovascular effects of splenomegaly and splenectomy in ‚- thalassemia. Ann Hematol 2005; 84:353-7.
  13. Selmi H, Khila M, Khlif A, Nabli S, Essoussi AS. Evaluation des résultats de la splénectomie partielle au cours de la ‚-thalassémie homozygote. À propos de 27 cas. Rev Maghr Pédiatr 2001:241-5.
  14. Girot R. Thalassémies chez l'enfant. Pédiatrie, Paris: Encycl Méd Chir; 1999; 4-080-A-30.
  15. Adekile A, Akar N, Ayyash K, Saldanha W, Prakash B. Indications for splenectomy among children with sickle cell disease in Kuwait. Kuwait Med J 2002; 34:33-5.
  16. Svarch E, Nordet I, Valdés J, González A, Machin S, de la Torre E. Partial splenectomy in children with sickle cell disease. Haematologica 2003; 88:222-3
  17. Nouri A, de Montalembert M, Revillon Y, Girot R. Partial splenectomy in sickle cell syndromes. Arch Dis Child 1991; 66:1070-2.
  18. Kimura F, Ito H, Shimizu H, Togawa A, Otsuka M, Yoshidome H, Shimamura F, Kato A, Nukui Y, Ambiru S, Miyazaki M. Partial splenic embolization for the treatment of hereditary spherocytosis. Am J Roentgenol 2003; 181:1021-4.
  19. Petroianu A, Resende V, Da Silva RG. Late follow-up of patients submitted to subtotal splenectomy. Int J Surg 2006.
  20. Buys Roessingh AS, de Lagausie P, Rohrlich P, Berrebi D, Aigrain Y. Follow-up of partial splenectomy in children with hereditary spherocytosis. J Pediatr Surg 2002; 37:1459-63.
  21. Stoehr GA, Stauffer UG, Eber SW. Near-total splenectomy: a new technique for management of hereditary spherocytosis. Ann Surg 2005; 241:40-7.
  22. Al-Salem AH, Qaisaruddin S, Nasserullah Z, Al Dabbous I, Abu Srair H, Al Jam'a A. Splenectomy and acute splenic sequestration crises in sickle cell disease. Pediatr Surg Int 1995; 11:26-8.
  23. Habibi A, Khellaf M, Bachir D, Schaeffer A, Galactéros F,Godeau B. La séquestration splénique aiguë: une complication rare et grave des syndromes drépanocytaires majeurs de l'adulte. A propos d'un cas. Rev Med Inter 2005; 26:339-42.
  24. Kehila M, Khelif A, Kharrat H, Ennabli S, Abderrahim T. La splénectomie partielle au cours des thalassémies majeures (A propos de 19 cas). J Chir (Paris) 1994;131:99-103
  25. Canatan D, Zorlu M, Bayir N, Erturk C, Dorak A, Oguz N, Balta N, Karadogan C. Thrombosis after splenectomy in patients with thalassemia. Turk J Haemat 2001; 18:259-63.
  26. Cappellini MD, Robbiolo L, Bottasso BM, Coppola R, Fiorelli G, Mannucci PM. Venous thromboembolism and hypercoagulability in splenectomized patients with thalassaemia intermedia. Br J Haemat 2000; 111:467-73.
  27. Kunin N, Desjardins JF, Letoquart JP, La Gamma A, Lebois E, Mambrini A. Thrombose mésentérico-portale après splénectomie hématologique. J Chir 1996; 133:453-8.
  28. Petit P, Bret PM, Atri M, Hreno A, Casola G, Gianfelice D. Splenic vein thrombosis after splenectomy: frequency and role of imaging. Radiology 1994; 190:65-8.
  29. Hassn AM, Al-Fallouji MA, Ouf TI, Saad R. Portal vein thrombosis following splenectomy. Br J Surg 2000; 87:362-73.
  30. Vento S, Cainelli F, Cesario F. Infections and thalassaemia. The Lancet 2006; 6:226-33.
  31. Brigden ML, Pattullo A, Brown G. Pneumococcal vaccine administration associated with splenectomy: the need for improved education, documentation, and the use of a practical checklist. Am J Hematol 2000; 65:25-9.
  32. Condon RJ, Riley TV, Kelly H. Invasive meningococcal infection after splenectomy. BMJ 1994; 308:792-3.
  33. Chanet V, Lesens O, Laurichesse H, Beytout J. Infections chez l'adulte asplénique et prévention. Médecine et maladies infectieuses 2004; 34:493-8.
  34. Legrand A, Bignon A, Borel M, Zerbib P, Langlois J, Chambon JP, Lebuffe G, Vallet B. Prévention du risque infectieux postopératoire chez les patients splénectomisés. Ann Fr Anesth Reanim 2005; 24 :807-13.
  35. Wright J, Thomas P, Serjeant GR. Septicemia caused by salmonella infection: An overlooked complication of sickle cell disease. J Pediatr 1997; 130:394-9.
  36. Lortan JE. Management of asplenic patients. Br J Haematol 1993; 84:566-9
  37. Portier H, Beytout J. Antibiothérapie préventive pour le splénectomisé et l'asplénique. Recommandations récentes. Médecine et maladies infectieuses 2004; 34:S67-S68.