Advertisement

Pediatric Radiology

, Volume 39, Issue 1, pp 17–22 | Cite as

The spleen in the sickling disorders: an update

  • Rana Khatib
  • Raja Rabah
  • Sharada A. Sarnaik
Clinical Review

Abstract

In early life, patients with sickle cell disease (SCD) can have acute, life-threatening emergencies related to splenic hypofunction (overwhelming bacterial sepsis), as well as anemic crises from acute splenic sequestration because of sudden pooling of blood in the spleen. The landmark penicillin prophylaxis study in 1985 showed a remarkable decrease in mortality from sepsis in young children with SCD who were treated with oral penicillin prophylaxis compared to placebo. Since that study, newborns are screened for SCD and placed on oral penicillin prophylaxis in nearly all of the United States, as well as in other countries where the disease is highly prevalent. The previously described permanent, complete and nearly universal “autosplenectomy” emerging by late childhood or early adulthood is now challenged by recent findings of reversibility of splenic dysfunction by the antisickling drug hydroxyurea or by successful allogeneic stem cell transplantation, even in older patients. Imaging techniques for hypofunction of the spleen are the most commonly used modalities to guide the clinician in decisions regarding medical or surgical management.

Keywords

Spleen Sickle cell disease Hydroxyurea 

References

  1. 1.
    Herrick JB (2001) Peculiar elongated and sickle-shaped red blood corpuscles in a case of severe anemia. 1910. Yale J Biol Med 74:179–184PubMedGoogle Scholar
  2. 2.
    Pauling L, Itano HA, Singer SJ et al (1949) Sickle cell anemia, a molecular disease. Science 110:543–548PubMedCrossRefGoogle Scholar
  3. 3.
    Ataga KI, Cappellini MD, Rachmilewitz EA (2007) Beta-thalassaemia and sickle cell anaemia as paradigms of hypercoagulability. Br J Haematol 139:3–13PubMedCrossRefGoogle Scholar
  4. 4.
    Ataga KI, Key NS (2007) Hypercoagulability in sickle cell disease: new approaches to an old problem. Hematology Am Soc Hematol Educ Program 2007:91–96PubMedGoogle Scholar
  5. 5.
    Ataga KI, Orringer EP (2003) Hypercoagulability in sickle cell disease: a curious paradox. Am J Med 115:721–728PubMedCrossRefGoogle Scholar
  6. 6.
    Onwubalili JK (1983) Sickle cell disease and infection. J Infect 7:2–20PubMedCrossRefGoogle Scholar
  7. 7.
    Overturf G, Powars D (1980) Infections in sickle cell anemia: pathogenesis and control. Tex Rep Biol Med 40:283–292PubMedGoogle Scholar
  8. 8.
    William BM, Corazza GR (2007) Hyposplenism: a comprehensive review. Part I: basic concepts and causes. Hematology 12:1–13PubMedCrossRefGoogle Scholar
  9. 9.
    Pearson HA, Spencer RP, Cornelius EA (1969) Functional asplenia in sickle-cell anemia. N Engl J Med 281:923–926PubMedGoogle Scholar
  10. 10.
    Dickerhoff R (2002) Splenic sequestration in patients with sickle cell disease. Klin Padiatr 214:70–73PubMedCrossRefGoogle Scholar
  11. 11.
    Benjamin JT, McMillan CW, Komp DM (1979) Fatal splenic sequestration crisis. Va Med 106:679–680PubMedGoogle Scholar
  12. 12.
    Walterspiel JN, Rutledge JC, Bartlett BL (1984) Fatal acute splenic sequestration at 4 months of age. Pediatrics 73:507–508PubMedGoogle Scholar
  13. 13.
    Emond AM, Collis R, Darvill D et al (1985) Acute splenic sequestration in homozygous sickle cell disease: natural history and management. J Pediatr 107:201–206PubMedCrossRefGoogle Scholar
  14. 14.
    Grover R, Wethers DL (1990) Management of acute splenic sequestration crisis in sickle cell disease. J Assoc Acad Minor Phys 1:67–70PubMedGoogle Scholar
  15. 15.
    Al-Salem AH, Naserullah Z, Qaisaruddin S et al (1999) Splenic complications of the sickling syndromes and the role of splenectomy. J Pediatr Hematol Oncol 21:401–406PubMedCrossRefGoogle Scholar
  16. 16.
    Delatte SJ, Hebra A, Tagge EP et al (1999) Acute chest syndrome in the postoperative sickle cell patient. J Pediatr Surg 34:188–191; discussion 191–192PubMedCrossRefGoogle Scholar
  17. 17.
    Giebink GS, Le CT, Schiffman G (1984) Decline of serum antibody in splenectomized children after vaccination with pneumococcal capsular polysaccharides. J Pediatr 105:576–582PubMedCrossRefGoogle Scholar
  18. 18.
    Wethers DL, Grover R (1987) Reversibility of splenic function by transfusion in two young adults with sickle cell anemia. Am J Pediatr Hematol Oncol 9:209–211PubMedGoogle Scholar
  19. 19.
    Pearson HA, Cornelius EA, Schwartz AD et al (1970) Transfusion-reversible functional asplenia in young children with sickle-cell anemia. N Engl J Med 283:334–337PubMedGoogle Scholar
  20. 20.
    Buchanan GR, McKie V, Jackson EA et al (1989) Splenic phagocytic function in children with sickle cell anemia receiving long-term hypertransfusion therapy. J Pediatr 115:568–572PubMedCrossRefGoogle Scholar
  21. 21.
    Koren A, Haasz R, Tiatler A et al (1984) Serum immunoglobulin levels in children after splenectomy. A prospective study. Am J Dis Child 138:53–55PubMedGoogle Scholar
  22. 22.
    Shatney CH (1987) Complications of splenectomy. Acta Anaesthesiol Belg 38:333–339PubMedGoogle Scholar
  23. 23.
    Barrett DJ, Ammann AJ (1981) Pneumococcal vaccine in sickle cell disease: IgG and IgM antibody response. Rev Infect Dis 3 [Suppl]:S179–S182PubMedGoogle Scholar
  24. 24.
    Risdon RA, Barry M, Flynn DM (1975) Transfusional iron overload: the relationship between tissue iron concentration and hepatic fibrosis in thalassaemia. J Pathol 116:83–95PubMedCrossRefGoogle Scholar
  25. 25.
    Campbell PJ, Olatunji PO, Ryan KE et al (1997) Splenic regrowth in sickle cell anaemia following hypertransfusion. Br J Haematol 96:77–79PubMedCrossRefGoogle Scholar
  26. 26.
    Wright JG, Hambleton IR, Thomas PW et al (1999) Postsplenectomy course in homozygous sickle cell disease. J Pediatr 134:304–309PubMedCrossRefGoogle Scholar
  27. 27.
    Vichinsky E, Pakbaz Z, Onyekwere O et al (2008) Patient-reported outcomes of deferasirox (Exjade, ICL670) versus deferoxamine in sickle cell disease patients with transfusional hemosiderosis. Substudy of a randomized open-label phase II trial. Acta Haematol 119:133–141PubMedCrossRefGoogle Scholar
  28. 28.
    Wood JC, Ghurge N (2008) Magnetic resonance imaging assessment of excess iron in thalassemia, sickle cell disease and other iron overload diseases. Hemoglobin 32:85–96PubMedCrossRefGoogle Scholar
  29. 29.
    Positano V, Salani B, Pepe A et al (2008) Improved T2* assessment in liver iron overload by magnetic resonance imaging. Magn Reson Imaging (in press) doi: 10.1016/j.mri.2008.06.004
  30. 30.
    Walker TM, Serjeant GR (1993) Focal echogenic lesions in the spleen in sickle cell disease. Clin Radiol 47:114–116PubMedCrossRefGoogle Scholar
  31. 31.
    Levin TL, Berdon WE, Haller JO et al (1996) Intrasplenic masses of “preserved” functioning splenic tissue in sickle cell disease: correlation of imaging findings (CT, ultrasound, MRI, and nuclear scintigraphy). Pediatr Radiol 26:646–649PubMedCrossRefGoogle Scholar
  32. 32.
    Casper JT, Koethe S, Rodey GE et al (1976) A new method for studying splenic reticuloendothelial dysfunction in sickle cell disease patients and its clinical application: a brief report. Blood 47:183–188PubMedGoogle Scholar
  33. 33.
    Santos A, Pinheiro V, Anjos C et al (2002) 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 29:536–541PubMedCrossRefGoogle Scholar
  34. 34.
    Hankins JS, Helton KJ, McCarville MB et al (2008) Preservation of spleen and brain function in children with sickle cell anemia treated with hydroxyurea. Pediatr Blood Cancer 50:293–297PubMedCrossRefGoogle Scholar
  35. 35.
    Wang WC, Wynn LW, Rogers ZR et al (2001) A two-year pilot trial of hydroxyurea in very young children with sickle-cell anemia. J Pediatr 139:790–796PubMedCrossRefGoogle Scholar
  36. 36.
    Claster S, Vichinsky E (1996) First report of reversal of organ dysfunction in sickle cell anemia by the use of hydroxyurea: splenic regeneration. Blood 88:1951–1953PubMedGoogle Scholar
  37. 37.
    Olivieri NF, Vichinsky EP (1998) Hydroxyurea in children with sickle cell disease: impact on splenic function and compliance with therapy. J Pediatr Hematol Oncol 20:26–31PubMedCrossRefGoogle Scholar
  38. 38.
    Ferster A, Bujan W, Corazza F et al (1993) Bone marrow transplantation corrects the splenic reticuloendothelial dysfunction in sickle cell anemia. Blood 81:1102–1105PubMedGoogle Scholar

Copyright information

© Springer-Verlag 2008

Authors and Affiliations

  1. 1.Children’s Hospital of Michigan, Carmen and Ann Adams Department of PediatricsWayne State University School of MedicineDetroitUSA
  2. 2.Department of PathologyWayne State University School of MedicineDetroitUSA

Personalised recommendations