Skip to main content
SpringerLink
Log in

Splenectomy in children with sickle cell disease and thalassemia

  • Original Articles
  • Published:
The Indian Journal of Pediatrics Aims and scope Submit manuscript

Abstract

A number of Saudi children (31) with sickle cell disease and thalassemia underwent splenectomy : 12 for frequent blood transfusions, 15 for chronic hypersplenism (most of whom were also the recipients of periodic blood transfusion) and 4 for splenic abscess. The mean age of splenectomy was 8.8 years (8 months 3–18 years). Eight patients had sickle cell disease, 14 betathalassemia and 9 had sickle cell thalassemia. All patients received prophylaxis against pneumococcal infection. There was one postoperative death most probably due to sepsis. Sixteen of those who required frequent preoperative blood transfusions needed no more transfusions, while in 7 the need for transfusions decreased significantly (p < 0.05). For those with hypersplenism, there was a significant postoperative increase in total hemoglobin (P < 0.001), RBC (P < 0.001) and platelet counts (p < 0.02); and a substantial decrease in reticulocyte counts (p < 0.05). The common post splenectomy complications were chest infection and a brief episode of pyrexia, but without undue morbidity. The study establishes a definite place for splenectomy in a selected population of children with sickle cell disease and thalassemia.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Al-Awamy BH. Sickle cell anemia: its clinical manifestations and their management.Saudi Medical Journal 1987; 9 : 553–562.

    Google Scholar 

  2. Pembrey ME. Sickle cell disease in Arabia. Postgraduate Doctor.Middle East 1980; June : 202–207.

  3. Perrine RP, Pembrey ME, John P et al. Natural history of sickle cell anaemia in Saudi Arabs.Ann Intern Med 1978; 88: 1–6.

    PubMed  CAS  Google Scholar 

  4. Pembrey ME, Weatherall DJ, Clegg JB et al. Haemoglobin Barts in Saudi Arabia.Br J Haematol 1975; 29: 221–230.

    PubMed  CAS  Google Scholar 

  5. Al-Awamy BH, Naeem MA. Sickle cell trait-its origin, prevalence and clinical implications.Saudi Medical Journal 1987; 8: 563–574.

    Google Scholar 

  6. Scher KS, Wroczynski AF, Joncs CW. Protection from post-splenectomy sepsis: effect of prophylactic penicillin and pneumococcal vaccine on clearance of type 3 pneumococcus.Surgery 1983; 93 : 792–797.

    PubMed  CAS  Google Scholar 

  7. Castaneda-Zuniga WR, Hammerschmidt DE, Sanchez R, Amplatzk. Non-surgical splenectomy.Am J Roenlgenol 1977; 129: 805–811.

    CAS  Google Scholar 

  8. Pringle KC, Spigos DG, Tan WS et al. Partial splenic embolization in the management of thalassemia major.J Pediat Surg 1982; 17: 884–891.

    Article  PubMed  CAS  Google Scholar 

  9. Spigos DG, Jonasson O, Mozes M, Caped V. Partial splenic embolization in the treatment of hypersplenism.American Journal of Radiology 1979, 132: 777–782.

    CAS  Google Scholar 

  10. Stellin G, Kumpe DA, Lilly JR. Splenic embolization in a child with hypersplenism.J Pediat Surg 1982; 17: 892–893.

    Article  PubMed  CAS  Google Scholar 

  11. Kherapdir MH, Alebouyeh M. Partial splenectomy in the treatment of thalassemia major.Z Kinderchir 1985; 40: 195–198.

    Google Scholar 

  12. DiCataldo A, Puleo S, LiDestri G et al. Splenic trauma and overwhelming postsplenectomy infection.Br J Surg 1987; 74: 343–345.

    Article  PubMed  Google Scholar 

  13. Schwartz PE, Sterioff S, Mucha P et al. Postsplenectomy sepsis and mortality in adults.JAMA 1982; 248: 2279–2283.

    Article  PubMed  CAS  Google Scholar 

  14. William WJ, Bentler E, Erslev AJ, Lichtman MA.Hematology, 3rd edition, New York, McGraw-Hill; 1983: 1538–1544.

    Google Scholar 

  15. Grant CS, Al-Salem A, Khwaja MS et al. Splenic abscess in children: aspects of management.Journal of the Royal College of Surgeons of Edinburgh 1987; 32 : 342–345.

    PubMed  CAS  Google Scholar 

  16. Emond AM, Venugopal S, Morais P et al. Role of splenectomy in homozygous sickle cell disease in childhood.Lancet 1984; i: 88–90.

    Article  Google Scholar 

  17. Craig RP, Bate CM, Humphries G. Some surgical aspects of homozygous betathalassemia.Br J Surg 1977; 64: 277–280.

    Article  PubMed  CAS  Google Scholar 

  18. Coon WW. Splenectomy in the treatment of hemolytic anemia.Arch Surg 1985; 120: 625–628.

    PubMed  CAS  Google Scholar 

  19. Angastiniotis MA, Hadjiminas MG. Thalassemia.Postgraduate Doctor — Middle East, 1984; December : 756–763.

  20. Bickerstaff KI, Morris PJ. Splenectomy for massive splenomegaly.Br J Sury 1987; 74: 346–349.

    Article  CAS  Google Scholar 

  21. Musser G. Lazer G, Hocking W, Busttil RW. Splenectomy for hemolytic disease. The UCLA experience with 306 patients.Ann Smg 1984; 200:40–45.

    CAS  Google Scholar 

  22. Holme TC, Crosby DL. Elective spleneÇtomy Indications and complications in 102 patients..Journal of the Royal College of Surgeons of Edinburgh 1984; 21: 229–231.

    Google Scholar 

  23. Rudowski WJ. Accessory spleens: clinical significance with particular reference to the recurrence of idiopathic thrombocytopenic purpura.World J of Surg 1985 ; 9 : 422–430.

    Article  CAS  Google Scholar 

  24. King H, Shumacker HB Jr. Splenic studies. Susceptibility to infection after splenectomy performed in infancy.Ann Surg 1952; 136: 239–242.

    Article  PubMed  CAS  Google Scholar 

  25. Walker W. Splenectomy in childhood. A review in England and Wales, 1960-4.Br J Surg 1976; 63: 36–43.

    Article  PubMed  CAS  Google Scholar 

  26. Philippart A, Hight DW. Splenectomy in childhood. Altered concepts of management.The American Journal of Pediatric I Ianatology Oncology 1980; 2: 62–68.

    Google Scholar 

  27. Dawson AA, Jones PF, King DG. Splcncclomy in the management of haematological disease.Br J Surg 1987; 74: 353–357.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Author notes

  1. M. S. Khwaja & C. Grant

    Present address: Departments of Surgery and Pediatrics, King Fahad Hospital of the University, P.O. Box 2208, 31952, Al-Khobar, Saudi Arabia

  2. M. Al-Fadel & B. Al Awami

    Present address: King Fahad Hospital of the University, P.O. Box 2208, 31952, Al-Khobar, Saudi Arabia

Authors and Affiliations

  1. Departments of Surgery and Pediatrics, King Fahad Hospital of the University, P.O. Box 2208, 31952, Al-Khobar, Saudi Arabia

    A. H. Al-Salem

Authors
  1. A. H. Al-Salem
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. M. S. Khwaja
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. M. Al-Fadel
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. C. Grant
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. B. Al Awami
    View author publications

    You can also search for this author in PubMed Google Scholar

Additional information

Presented at the 9th Congress of the Asian Association of Pediatric Surgeons, April 6–10, 1988, Singapore.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Al-Salem, A.H., Khwaja, M.S., Al-Fadel, M. et al. Splenectomy in children with sickle cell disease and thalassemia. Indian J Pediatr 56, 747–752 (1989). https://doi.org/10.1007/BF02724458

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02724458

Key Words

Search

Navigation