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. 1990 Mar;65(3):304–307. doi: 10.1136/adc.65.3.304

Partial splenectomy in homozygous beta thalassaemia.

M de Montalembert 1, R Girot 1, Y Revillon 1, D Jan 1, L Adjrad 1, F Z Ardjoun 1, M Belhani 1, Y Najean 1
PMCID: PMC1792263  PMID: 2334210

Abstract

Partial splenectomy was performed on 30 patients with homozygous beta thalassaemia to reduce blood requirements and to avoid the risk of overwhelming postsplenectomy infections; 24 patients had thalassaemia major and six thalassaemia intermedia. Five patients received a high transfusion regimen before and after surgery and 25 a lower one. Follow up after surgery ranged from one to four years. Partial splenectomy improved the long term haematological state in the six patients with thalassaemia intermedia. Recurrence of hypersplenism occurred in nine of the 24 patients with thalassaemia major, however, and complete splenectomy was required. Serum IgM concentrations were not significantly modified by surgery. The mean (SD) residual spleen after surgery was 4.45 (2.36) cm measured by scintigraphy. No severe infections occurred after surgery; however, most patients were routinely treated with phenoxymethylpenicillin and the protective effect of the remaining spleen could not be exactly determined. Because of the possibility of recurrence of hypersplenism, routine partial splenectomy when splenectomy is needed in thalassaemia major is not advised, except in children under 5 years whose risk of overwhelming postsplenectomy infection is greatest.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 20-1983. Hyperthermia of acute onset in an asplenic man. N Engl J Med. 1983 May 19;308(20):1212–1218. doi: 10.1056/NEJM198305193082008. [DOI] [PubMed] [Google Scholar]
  2. Colonna P., Ardjoun F. Z. Oral penicillin prophylaxis in thalassemia and in sickle cell anemia. N Engl J Med. 1986 Nov 6;315(19):1230–1230. doi: 10.1056/NEJM198611063151917. [DOI] [PubMed] [Google Scholar]
  3. ERLANDSON M. E., BRILLIANT R., SMITH C. H. COMPARISON OF SIXTY-SIX PATIENTS WITH THALASSEMIA MAJOR AND THIRTEEN PATIENTS WITH THALASSEMIA INTERMEDIA: INCLUDING EVALUATIONS OF GROWTH, DEVELOPMENT, MATURATION AND PROGNOSIS. Ann N Y Acad Sci. 1964 Oct 7;119:727–735. doi: 10.1111/j.1749-6632.1965.tb54074.x. [DOI] [PubMed] [Google Scholar]
  4. Graziano J. H., Piomelli S., Hilgartner M., Giardina P., Karpatkin M., Andrew M., LoIacono N., Seaman C. Chelation therapy in beta-thalassemia major. III. The role of splenectomy in achieving iron balance. J Pediatr. 1981 Nov;99(5):695–699. doi: 10.1016/s0022-3476(81)80386-1. [DOI] [PubMed] [Google Scholar]
  5. Heier H. E. Splenectomy and serious infections. Scand J Haematol. 1980 Jan;24(1):5–12. doi: 10.1111/j.1600-0609.1980.tb01311.x. [DOI] [PubMed] [Google Scholar]
  6. Kheradpir M. H., Alebouyeh M. Partial splenectomy in the treatment of thalassaemia major. Z Kinderchir. 1985 Aug;40(4):195–198. doi: 10.1055/s-2008-1059772. [DOI] [PubMed] [Google Scholar]
  7. Lockwood C. M. Immunological functions of the spleen. Clin Haematol. 1983 Jun;12(2):449–465. [PubMed] [Google Scholar]
  8. Modell B. Total management of thalassaemia major. Arch Dis Child. 1977 Jun;52(6):489–500. doi: 10.1136/adc.52.6.489. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Politis C., Spigos D. G., Georgiopoulou P., Vrettou H., Economidou I., Germenis A. E., Richardson C., Papaevangelou G. Partial splenic embolisation for hypersplenism of thalassaemia major: five year follow up. Br Med J (Clin Res Ed) 1987 Mar 14;294(6573):665–667. doi: 10.1136/bmj.294.6573.665. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Revillon Y., Girot R. Désartérialisation partielle de la rate et splénectomie partielle chez l'enfant. Presse Med. 1985 Feb 16;14(7):423–425. [PubMed] [Google Scholar]
  11. SMITH C. H., ERLANDSON M. E., STERN G., HILGARTNER M. W. POSTSPLENECTOMY INFECTION IN COOLEY'S ANEMIA. Ann N Y Acad Sci. 1964 Oct 7;119:748–757. doi: 10.1111/j.1749-6632.1965.tb54076.x. [DOI] [PubMed] [Google Scholar]

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