The Spleen and Sickle Cell Anemia

  • Chapter
  • First Online:
The Spleen
  • 137 Accesses

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

Access this chapter

Subscribe and save

Springer+ Basic
EUR 32.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or Ebook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 169.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 219.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free ship** worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Further Reading

  1. Al-Salem AH. Indications and complications of splenectomy for children with sickle cell disease. J Pediatr Surg. 2006;41:1909–15.

    Article  PubMed  Google Scholar 

  2. Haricharan RN, Roberts JM, Morgan TL, et al. Splenectomy reduces packed red cell transfusion requirements in children with sickle cell disease. J Pediatr Surg. 2008;43(6):1052–6.

    Article  PubMed  Google Scholar 

  3. Al Jam’a A, Al Salem AH, Al Dabous I. Massive splenic infarction in Saudi patients with sickle cell anemia: A unique manifestation. Am J Haematol. 2002;69:205–9.

    Article  Google Scholar 

  4. Al-Salem AH, Qaisaruddin S, Al Jam’a A, Al-Kalaf J, Al-Bashier AM. Splenic abscess and sickle cell disease. Am J Haematol. 1998;58:100–4.

    Article  CAS  Google Scholar 

  5. Al-Awamy BH, Niazi GA, El-Mouzan MI, et al. Relationship of haemoglobin F and alpha-thalassemia to severity of sickle cell anemia in the eastern province of Saudi Arabia. Ann Trop Pediatr. 1986;6:261–5.

    Article  CAS  Google Scholar 

  6. Elbashier AM, Al-Salem AH, Aljama A. Salmonella as a causative organism of various infections in patients with sickle cell disease. Ann Saudi Med. 2003;23:358–62.

    Article  PubMed  Google Scholar 

  7. Lerner RM, Spataro RF. Splenic abscess: percutaneous drainage. Radiology. 1984;153:643–5.

    Article  CAS  PubMed  Google Scholar 

  8. Fernandes ET, Tavares PB, Gaarcette CBM. Conservative management of splenic abscess in children. J Pediatr Surg. 1992;27:1578–9.

    Article  CAS  PubMed  Google Scholar 

  9. Bhattacharyya N, Ablin DS, Kosloske AM. Stapled partial splenectomy for splenic abscess in a child. J Pediatr Surg. 1989;24:316–7.

    Article  CAS  PubMed  Google Scholar 

  10. Platt O, Brambilla DJ, Roses WF, et al. Mortality in sickle cell disease: life expectancy and risk factors for early death. N Engl J Med. 1994;330:1639–44.

    Article  CAS  PubMed  Google Scholar 

  11. Pugliese R, Sansonna F, Scandroglio I, et al. Laparoscopic splenectomy: a retrospective review of 75 cases. Int Surg. 2006;91:82–6.

    CAS  PubMed  Google Scholar 

  12. Al-Wabari A, Parida L, Al-Salem AH. Laparoscopic splenectomy and/or cholecystectomy for children with sickle cell disease. Pediatr Surg Int. 2009;25(5):417–21.

    Article  Google Scholar 

  13. Waghorn DJ. Overwhelming infection in asplenic patients: current best practice preventive measures are not being followed. J Clin Pathol. 2001;54(3):214–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  14. Corcione F, Pirozzi F, Aragiusto G, Galante F, Sciuto A. Laparoscopic splenectomy: experience of a single center in a series of 300 cases. Surg Endosc. 2012;26(10):2870–6.

    Article  PubMed  Google Scholar 

  15. Al-Salem AH. Splenic complications of sickle cell anemia and the role of splenectomy. ISRN Hematol. 2011;2011:864257.

    PubMed  Google Scholar 

  16. Al-Salem AH. Massive splenic infarction in children with sickle cell anemia and the role of splenectomy. Pediatr Surg Int. 2013;29(3):281–5.

    Article  PubMed  Google Scholar 

  17. Al-Wabari A, Parida L, Al-Salem AH. Laparoscopic splenectomy for children with hematological disorders: our initial experience with 45 cases. J Pediatr Surg Specialt. 2008;2(3):18–20.

    Google Scholar 

  18. Airede AI. Acute splenic sequestration in a five-week-old infant with sickle cell disease. J Pediatr. 1992;120:160.

    Article  CAS  PubMed  Google Scholar 

  19. Alkindi S, Al-Maini M, Pathare A. Clinical and laboratory characteristics of patients with sickle-cell and autoimmune/connective tissue diseases. Rheumatol Int. 2012;32:373–8.

    Article  PubMed  Google Scholar 

  20. Al-Salem AH, Al-Aithan S, Bhamidipati P, Al-Jam’a A, Al Dabbous I. Sonographic assessment of spleen size in Saudi patients with sickle cell disease. Ann Saudi Med. 1998;18:217–20.

    Article  CAS  PubMed  Google Scholar 

  21. Ammann AJ, Addiego J, Wara DW, Lubin B, Smith WB, Mentzer WC. Polyvalent pneumococcal-polysaccharide immunization of patients with sickle-cell anemia and patients with splenectomy. N Engl J Med. 1977;297:897–900.

    Article  CAS  PubMed  Google Scholar 

  22. Aneni EC, Hamer DH, Gill CJ. Systematic review of current and emerging strategies for reducing morbidity from malaria in sickle cell disease. Tropical Med Int Health. 2013;18:313–27.

    Article  CAS  Google Scholar 

  23. Aquino VM, Norvell JM, Buchanan GR. Acute splenic complications in children with sickle cell-hemoglobin C disease. J Pediatr. 1997;130:961–5.

    Article  CAS  PubMed  Google Scholar 

  24. Baethge BA, Bordelon TR, Mills GM, Bowen LM, Wolf RE, Bairnsfather L. Antinuclear antibodies in sickle cell disease. Acta Haematol. 1990;84:186–9.

    Article  CAS  PubMed  Google Scholar 

  25. Barrett-Connor E. Bacterial infection and sickle cell anemia. An analysis of 250 infections in 166 patients and a review of the literature. Medicine. 1971;50:97–112.

    Article  CAS  PubMed  Google Scholar 

  26. Barrios NJ, Livaudais F, McNeil J, Humbert JR, Corrigan J. Reversible splenic hypofunction in hypertransfused children with homozygous sickle cell disease. J Natl Med Assoc. 1993;85:677–80.

    CAS  PubMed  PubMed Central  Google Scholar 

  27. Brousse V, Elie C, Benkerrou M, Odièvre MH, Lesprit E, Bernaudin F, Grimaud M, Guitton C, Quinet B, Dangiolo S, de Montalembert M. Acute splenic sequestration crisis: cohort of 190 paediatric patients. Br J Haematol. 2012;156:643–8.

    Article  PubMed  Google Scholar 

  28. Brown AK, Sleeper LA, Miller ST, Pegelow CH, Gill FM, Waclawiw MA. Reference values and hematologic changes from birth to 5 years in patients with sickle cell disease. Cooperative Study of Sickle Cell Disease. Arch Pediatr Adolesc Med. 1994;148:796–804.

    Article  CAS  PubMed  Google Scholar 

  29. Buffet PA, Milon G, Brousse V, Correas JM, Dousset B, Couvelard A, Kianmanesh R, Farges O, Sauvanet A, Paye F, Ungeheuer MN, Ottone C, Khun H, Fiette L, Guigon G, Huerre M, Mercereau-Puijalon O, David PH. Ex vivo perfusion of human spleens maintains clearing and processing functions. Blood. 2006;107:3745–52.

    Article  CAS  PubMed  Google Scholar 

  30. Cameron PU, Jones P, Gorniak M, Dunster K, Paul E, Lewin S, Woolley I, Spelman D. Splenectomy associated changes in IgM memory B cells in an adult spleen registry cohort. PLoS One. 2011;6:e23164.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  31. Campbell PJ, Olatunji PO, Ryan KE, Davies SC. Splenic regrowth in sickle cell anaemia following hypertransfusion. Br J Haematol. 1997;96:77–9.

    Article  CAS  PubMed  Google Scholar 

  32. Chen LT, Weiss L. Electron microscopy of the red pulp of human spleen. Am J Anat. 1972;134:425–57.

    Article  CAS  PubMed  Google Scholar 

  33. Crary SE, Buchanan GR. Vascular complications after splenectomy for hematologic disorders. Blood. 2009;114:2861–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  34. Deplaine G, Safeukui I, Jeddi F, Lacoste F, Brousse V, Perrot S, Biligui S, Guillotte M, Guitton C, Dokmak S, Aussilhou B, Sauvanet A, Cazals Hatem D, Paye F, Thellier M, Mazier D, Milon G, Mohandas N, Mercereau-Puijalon O, David PH, Buffet PA. The sensing of poorly deformable red blood cells by the human spleen can be mimicked in vitro. Blood. 2011;117:e88–95.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  35. Di Sabatino A, Carsetti R, Corazza GR. Post-splenectomy and hyposplenic states. Lancet. 2011;378:86–97.

    Article  PubMed  Google Scholar 

  36. Diggs LW. Siderofibrosis of the spleen in sickle cell anemia. JAMA. 1935;104:538–41.

    Article  Google Scholar 

  37. Donald DE, Aarhus LL. Active and passive release of blood from canine spleen and small intestine. Am J Physiol. 1974;227:1166–72.

    Article  CAS  PubMed  Google Scholar 

  38. El Nemer W, Gane P, Colin Y, Bony V, Rahuel C, Galacteros F, Cartron JP, Le Van Kim C. The Lutheran blood group glycoproteins, the erythroid receptors for laminin, are adhesion molecules. J Biol Chem. 1998;273:16686–93.

    Article  PubMed  Google Scholar 

  39. Emond AM, Collis R, Darvill D, Higgs DR, Maude GH, Serjeant GR. Acute splenic sequestration in homozygous sickle cell disease: natural history and management. J Pediatr. 1985;107:201–6.

    Article  CAS  PubMed  Google Scholar 

  40. Ferster A, Bujan W, Corazza F, Devalck C, Fondu P, Toppet M, Verhas M, Sariban E. Bone marrow transplantation corrects the splenic reticuloendothelial dysfunction in sickle cell anemia. Blood. 1993;81:1102–5.

    Article  CAS  PubMed  Google Scholar 

  41. Fontana V, Jy W, Ahn ER, Dudkiewicz P, Horstman LL, Duncan R, Ahn YS. Increased procoagulant cell-derived microparticles (C-MP) in splenectomized patients with ITP. Thromb Res. 2008;122:599–603.

    Article  CAS  PubMed  Google Scholar 

  42. Gaston MH, Verter JI, Woods G, Pegelow C, Kelleher J, Presbury G, Zarkowsky H, Vichinsky E, Iyer R, Lobel JS, Diamond S, Holbrook CT, Gill FM, Richey K, Faletta JM, for the Prophylactic Penicillin Study Group. Prophylaxis with oral penicillin in children with sickle cell anemia. A randomized trial. N Engl J Med. 1986;314:1593–9.

    Article  CAS  PubMed  Google Scholar 

  43. Gladwin MT, Sachdev V, Jison ML, Shizukuda Y, Plehn JF, Minter K, Brown B, Coles WA, Nichols JS, Ernst I, Hunter LA, Blackwelder WC, Schechter AN, Rodgers GP, Castro O, Ognibene FP. Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med. 2004;350:886–95.

    Article  CAS  PubMed  Google Scholar 

  44. Gotthardt M, Broker S, Schlieck A, Bauhofer A, Herbst B, Behe M, Corstens FH, Behr TM, Gorg C. Scintigraphy with 99mTc-labeled heat-altered erythrocytes in diagnosing hyposplenia: prospective comparison to 99mTc-labeled colloids and colour-coded duplex ultrasonography. Nuklearmedizin. 2007;46:135–40.

    Article  CAS  PubMed  Google Scholar 

  45. den Haan JM, Kraal G. Innate immune functions of macrophage subpopulations in the spleen. J Innate Immun. 2012;4:437–45.

    Article  Google Scholar 

  46. 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;35:179–83.

    Article  PubMed  Google Scholar 

  47. Higgs DR, Aldridge BE, Lamb J, Clegg JB, Weatherall DJ, Hayes RJ, Grandison Y, Lowrie Y, Mason KP, Serjeant BE, Serjeant GR. The interaction of alpha-thalassemia and homozygous sickle-cell disease. N Engl J Med. 1982;306:1441–6.

    Article  CAS  PubMed  Google Scholar 

  48. Hirst C, Owusu-Ofori S. Prophylactic antibiotics for preventing pneumococcal infection in children with sickle cell disease. Cochrane Database Syst Rev. 2012;9:CD003427.

    Google Scholar 

  49. Holroyde CP, Oski FA, Gardner FH. The “pocked” erythrocyte. Red-cell surface alterations in reticuloendothelial immaturity of the neonate. N Engl J Med. 1969;281:516–20.

    Article  CAS  PubMed  Google Scholar 

  50. Imbert P, Rapp C, Buffet PA. Pathological rupture of the spleen in malaria: analysis of 55 cases (1958–2008). Travel Med Infect Dis. 2009;7:147–59.

    Article  PubMed  Google Scholar 

  51. 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;26:495–500.

    Article  PubMed  Google Scholar 

  52. Kato GJ, Hebbel RP, Steinberg MH, Gladwin MT. Vasculopathy in sickle cell disease: biology, pathophysiology, genetics, translational medicine, and new research directions. Am J Hematol. 2009;84:618–25.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  53. Kinney TR, Ware RE, Schultz WH, Filston HC. Long-term management of splenic sequestration in children with sickle cell disease. J Pediatr. 1990;117:194–9.

    Article  CAS  PubMed  Google Scholar 

  54. Koduri PR, Nathan S. Acute splenic sequestration crisis in adults with hemoglobin S-C disease: a report of nine cases. Ann Hematol. 2006;85:239–43.

    Article  PubMed  Google Scholar 

  55. Komba AN, Makani J, Sadarangani M, Ajala-Agbo T, Berkley JA, Newton CR, Marsh K, Williams TN. Malaria as a cause of morbidity and mortality in children with homozygous sickle cell disease on the coast of Kenya. Clin Infect Dis. 2009;49:216–22.

    Article  PubMed  Google Scholar 

  56. Lane PA, O’Connell JL, Lear JL, Rogers ZR, Woods GM, Hassell KL, Wethers DL, Luckey DW, Buchanan GR. Functional asplenia in hemoglobin SC disease. Blood. 1995;85:2238–44.

    Article  CAS  PubMed  Google Scholar 

  57. Lee A, Thomas P, Cupidore L, Serjeant B, Serjeant G. Improved survival in homozygous sickle cell disease: lessons from a cohort study. BMJ. 1995;311:1600–2.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  58. Lesher AP, Kalpatthi R, Glenn JB, Jackson SM, Hebra A. Outcome of splenectomy in children younger than 4 years with sickle cell disease. J Pediatr Surg. 2009;44:1134–8. discussion 1138

    Article  PubMed  Google Scholar 

  59. Makani J, Komba AN, Cox SE, Oruo J, Mwamtemi K, Kitundu J, Magesa P, Rwezaula S, Meda E, Mgaya J, Pallangyo K, Okiro E, Muturi D, Newton CR, Fegan G, Marsh K, Williams TN. Malaria in patients with sickle cell anemia: burden, risk factors, and outcome at the outpatient clinic and during hospitalization. Blood. 2010;115:215–20.

    Article  CAS  PubMed  Google Scholar 

  60. McAuley CF, Webb C, Makani J, Macharia A, Uyoga S, Opi DH, Ndila C, Ngatia A, Scott JA, Marsh K, Williams TN. High mortality from Plasmodium falciparum malaria in children living with sickle cell anemia on the coast of Kenya. Blood. 2010;116:1663–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  61. McCarville MB, Luo Z, Huang X, Rees RC, Rogers ZR, Miller ST, Thompson B, Kalpatthi R, Wang WC. Abdominal ultrasound with scintigraphic and clinical correlates in infants with sickle cell anemia: baseline data from the BABY HUG trial. AJR Am J Roentgenol. 2011;196:1399–404.

    Article  PubMed  PubMed Central  Google Scholar 

  62. McCavit TL, Quinn CT, Techasaensiri C, Rogers ZR. Increase in invasive Streptococcus pneumoniae infections in children with sickle cell disease since pneumococcal conjugate vaccine licensure. J Pediatr. 2011;158:505–7.

    Article  PubMed  Google Scholar 

  63. Naik RP, Streiff MB, Haywood C Jr, Nelson JA, Lanzkron S. Venous thromboembolism in adults with sickle cell disease: a serious and under-recognized complication. Am J Med. 2013;126:443–9.

    Article  PubMed  PubMed Central  Google Scholar 

  64. Ohene-Frempong K, Weiner SJ, Sleeper LA, Miller ST, Embury S, Moohr JW, Wethers DL, Pegelow CH, Gill FM. Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood. 1998;91:288–94.

    CAS  PubMed  Google Scholar 

  65. Owusu-Ofori S, Hirst C. Splenectomy versus conservative management for acute sequestration crises in people with sickle cell disease. Cochrane Database Syst Rev. 2013;5:CD003425.

    Google Scholar 

  66. Parent F, Bachir D, Inamo J, Lionnet F, Driss F, Loko G, Habibi A, Bennani S, Savale L, Adnot S, Maitre B, Yaici A, Hajji L, O’Callaghan DS, Clerson P, Girot R, Galacteros F, Simonneau G. A hemodynamic study of pulmonary hypertension in sickle cell disease. N Engl J Med. 2011;365:44–53.

    Article  CAS  PubMed  Google Scholar 

  67. Payne AB, Link-Gelles R, Azonobi I, Hooper WC, Beall BW, Jorgensen JH, Juni B, Moore M. Invasive pneumococcal disease among children with and without sickle cell disease in the United States, 1998–2009. Pediatr Infect Dis J. 2013;32:1308–12.

    Article  PubMed  PubMed Central  Google Scholar 

  68. Pearson HA, Spencer RP, Cornelius EA. Functional asplenia in sickle-cell anemia. N Engl J Med. 1969;281:923–6.

    Article  CAS  PubMed  Google Scholar 

  69. Pearson HA, Cornelius EA, Schwartz AD, Zelson JH, Wolfson SL, Spencer RP. Transfusion-reversible functional asplenia in young children with sickle-cell anemia. N Engl J Med. 1970;283:334–7.

    Article  CAS  PubMed  Google Scholar 

  70. Pearson HA, Gallagher D, Chilcote R, Sullivan E, Wilimas J, Espeland M, Ritchey AK. Developmental pattern of splenic dysfunction in sickle cell disorders. Pediatrics. 1985;76:392–7.

    Article  CAS  PubMed  Google Scholar 

  71. Piccin A, Rizkalla H, Smith O, McMahon C, Furlan C, Murphy C, Negri G, Mc Dermott M. Composition and significance of splenic Gamna-Gandy bodies in sickle cell anemia. Hum Pathol. 2012;43:1028–36.

    Article  CAS  PubMed  Google Scholar 

  72. van der Plas EM, van den Tweel XW, Geskus RB, Heijboer H, Biemond BJ, Peters M, Fijnvandraat K. Mortality and causes of death in children with sickle cell disease in the Netherlands, before the introduction of neonatal screening. Br J Haematol. 2011;155:106–10.

    Article  PubMed  Google Scholar 

  73. Powell RW, Levine GL, Yang YM, Mankad VN. Acute splenic sequestration crisis in sickle cell disease: early detection and treatment. J Pediatr Surg. 1992;27:215–8; discussion 218–219

    Article  CAS  PubMed  Google Scholar 

  74. Quinn CT, Rogers ZR, McCavit TL, Buchanan GR. Improved survival of children and adolescents with sickle cell disease. Blood. 2010;115:3447–52.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  75. Ram S, Lewis LA, Rice PA. Infections of people with complement deficiencies and patients who have undergone splenectomy. Clin Microbiol Rev. 2010;23:740–80.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  76. Rescorla FJ, West KW, Engum SA, Grosfeld JL. Laparoscopic splenic procedures in children: experience in 231 children. Ann Surg. 2007;246:683–7; discussion 687–688

    Article  PubMed  Google Scholar 

  77. Rice TW, Rubinson L, Uyeki TM, Vaughn FL, John BB, Miller RR 3rd, Higgs E, Randolph AG, Smoot BE, Thompson BT. Critical illness from 2009 pandemic influenza A virus and bacterial coinfection in the United States. Crit Care Med. 2012;40:1487–98.

    Article  PubMed  PubMed Central  Google Scholar 

  78. Rogers ZR, Wang WC, Luo Z, Iyer RV, Shalaby-Rana E, Dertinger SD, Shulkin BL, Miller JH, Files B, Lane PA, Thompson BW, Miller ST, Ware RE. Biomarkers of splenic function in infants with sickle cell anemia: baseline data from the BABY HUG trial. Blood. 2011;117:2614–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  79. Rosado MM, Gesualdo F, Marcellini V, Di Sabatino A, Corazza GR, Smacchia MP, Nobili B, Baronci C, Russo L, Rossi F, Vito RD, Nicolosi L, Inserra A, Locatelli F, Tozzi AE, Carsetti R. Preserved antibody levels and loss of memory B cells against pneumococcus and tetanus after splenectomy: tailoring better vaccination strategies. Eur J Immunol. 2013;43:2659–70.

    Article  CAS  PubMed  Google Scholar 

  80. Sadarangani M, Makani J, Komba AN, Ajala-Agbo T, Newton CR, Marsh K, Williams TN. An observational study of children with sickle cell disease in Kilifi, Kenya. Br J Haematol. 2009;146:675–82.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  81. Safeukui I, Correas JM, Brousse V, Hirt D, Deplaine G, Mule S, Lesurtel M, Goasguen N, Sauvanet A, Couvelard A, Kerneis S, Khun H, Vigan-Womas I, Ottone C, Molina TJ, Treluyer JM, Mercereau-Puijalon O, Milon G, David PH, Buffet PA. Retention of Plasmodium falciparum ring-infected erythrocytes in the slow, open microcirculation of the human spleen. Blood. 2008;112:2520–8.

    Article  CAS  PubMed  Google Scholar 

  82. Serjeant GR. The spleen in sickle cell disease. In: Bowdler AJ, editor. The complete spleen: a handbook of structure, function, and clinical disorders. Totowa, NJ: Humana Press; 2001. p. 251–7.

    Google Scholar 

  83. Stein PD, Beemath A, Meyers FA, Skaf E, Olson RE. Deep venous thrombosis and pulmonary embolism in hospitalized patients with sickle cell disease. Am J Med. 2006;119(897):e7–11.

    Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  85. Telfer P, Coen P, Chakravorty S, Wilkey O, Evans J, Newell H, Smalling B, Amos R, Stephens A, Rogers D, Kirkham F. Clinical outcomes in children with sickle cell disease living in England: a neonatal cohort in East London. Haematologica. 2007;92:905–12.

    Article  PubMed  Google Scholar 

  86. Topley JM, Rogers DW, Stevens MC, Serjeant GR. Acute splenic sequestration and hypersplenism in the first five years in homozygous sickle cell disease. Arch Dis Child. 1981;56:765–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  87. Wandersee NJ, Punzalan RC, Rettig MP, Kennedy MD, Pajewski NM, Sabina RL, Paul Scott J, Low PS, Hillery CA. Erythrocyte adhesion is modified by alterations in cellular tonicity and volume. Br J Haematol. 2005;131:366–77.

    Article  CAS  PubMed  Google Scholar 

  88. Wang WC, Wynn LW, Rogers ZR, Scott JP, Lane PA, Ware RE. A two-year pilot trial of hydroxyurea in very young children with sickle-cell anemia. J Pediatr. 2001;139:790–6.

    Article  CAS  PubMed  Google Scholar 

  89. Wang WC, Ware RE, Miller ST, Iyer RV, Casella JF, Minniti CP, Rana S, Thornburg CD, Rogers ZR, Kalpatthi RV, Barredo JC, Brown RC, Sarnaik SA, Howard TH, Wynn LW, Kutlar A, Armstrong FD, Files BA, Goldsmith JC, Waclawiw MA, Huang X, Thompson BW. Hydroxycarbamide in very young children with sickle-cell anaemia: a multicentre, randomised, controlled trial (BABY HUG). Lancet. 2011;377:1663–72.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  90. Weller S, Braun MC, Tan BK, Rosenwald A, Cordier C, Conley ME, Plebani A, Kumararatne DS, Bonnet D, Tournilhac O, Tchernia G, Steiniger B, Staudt LM, Casanova JL, Reynaud CA, Weill JC. Human blood IgM “memory” B cells are circulating splenic marginal zone B cells harboring a prediversified immunoglobulin repertoire. Blood. 2004;104:3647–54.

    Article  CAS  PubMed  Google Scholar 

  91. WHO. Guidelines for the treatment of malaria. 2nd ed. Geneva: World Health Organization; 2010.

    Google Scholar 

  92. William BM, Corazza GR. Hyposplenism: a comprehensive review. Part I: basic concepts and causes. Hematology. 2007;12:1–13.

    Article  PubMed  Google Scholar 

  93. Wright JG, Hambleton IR, Thomas PW, Duncan ND, Venugopal S, Serjeant GR. Postsplenectomy course in homozygous sickle cell disease. J Pediatr. 1999;134:304–9.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

Copyright information

© 2023 The Author(s), under exclusive license to Springer Nature Singapore Pte Ltd.

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Al-Salem, A.H. (2023). The Spleen and Sickle Cell Anemia. In: The Spleen. Springer, Singapore. https://doi.org/10.1007/978-981-99-6191-7_21

Download citation

  • DOI: https://doi.org/10.1007/978-981-99-6191-7_21

  • Published:

  • Publisher Name: Springer, Singapore

  • Print ISBN: 978-981-99-6190-0

  • Online ISBN: 978-981-99-6191-7

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Navigation