eMedicine Specialties > Pediatrics: General Medicine > Allergy & Immunology

Asplenia: Follow-up

Author: Joseph C Turbyville, MD, Fellow, Department of Allergy and Immunology, Walter Reed Army Medical Center
Coauthor(s): Cecilia P Mikita, MD, MPH, Associate Program Director, Allergy-Immunology Fellowship, Chief, Clinical Services, Allergy-Immunology Clinic, Walter Reed Army Medical Center; Mudra Kumar, MD, MBBS, MRCP, Associate Professor, Department of Pediatrics, University of South Florida College of Medicine
Contributor Information and Disclosures

Updated: Aug 13, 2008

Follow-up

Further Inpatient Care

  • The most difficult and crucial aspect of asplenia is establishing the diagnosis.
    • Although this task is relatively simple in patients with accompanying anomalies, especially complex cyanotic cardiac problems, and in those with a family history of the condition, the patient with isolated asplenia or hyposplenia may not be easily identified.
    • The diagnosis is often made at autopsy.
  • Patients require regular monitoring with an established provider.
  • All immunizations, including routine childhood vaccinations and additional immunizations, are recommended (see Medical Care).
    • These vaccinations should be administered at the earliest opportunity.
    • Close observation and monitoring is mandatory, especially in the first few years of childhood, to educate the family and to ensure compliance with antibiotic prophylaxis.

Prognosis

  • With early diagnosis and aggressive treatment, the long-term prognosis of a child with isolated congenital asplenia is good.
    • The risk of overwhelming sepsis, although it does not end, significantly decreases in individuals older than 5 years.
    • The primary care physician plays an integral role in the identification and long-term treatment of patients with asplenia.
  • Congenital asplenia, polysplenia, and hypoplasia may be underdiagnosed. An increased awareness of their existence may be crucial and life saving in immunocompromised individuals with these conditions.

Patient Education

Miscellaneous

Medicolegal Pitfalls

  • Unfortunately, isolated asplenia or hyposplenia is often diagnosed at autopsy.
  • A high index of suspicion and increased awareness are important for the diagnosis.
 
Acknowledgments

Thanks to Oswaldo Castro, MD, for his assistance in reviewing this manuscript and providing expertise with regards to management of patients with sickle cell disease and asplenia.



More on Asplenia

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References
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References

  1. Fremont RD, Rice TW. Splenosis: a review. South Med J. Jun 2007;100(6):589-93. [Medline].

  2. Leahy RT, Philip RK, Gibbons RJ, et al. Asplenia in ATR-X Syndrome: a second report. Am J Med Genet A. 2005;15;139 (1):37-9. [Medline].

  3. Casey B, Devoto M, Jones KL, Ballabio A. Mapping a gene for familial situs abnormalities to human chromosome Xq24-q27.1. Nat Genet. Dec 1993;5(4):403-7. [Medline].

  4. US Preventive Services Task Force. Screening for sickle cell disease in newborns: recommendation statement. Am Fam Physician. May 1 2008;77(9):1300-2. [Medline].

  5. Committee on Infectious Diseases American Academy of Pediatrics. Red Book: 2006 Report of the Committee on Infectious Diseases. 27th Ed. 2006.

  6. Elsayes KM, Narra VR, Mukundan G, et al. MR imaging of the spleen: spectrum of abnormalities. Radiographics. 2005;Jul-Aug;25(4):967-82. [Medline]. [Full Text].

  7. Garson A, Bricker JT, McNamara DG. The Science and Practice of Pediatric Cardiology. Vol 3. 1990:1288-97.

  8. Gill DG, Kara M, Moore L. Archives of diseases in childhood. Vol 66. 1991:1366.

  9. Gorg C, Eickhorn M, Zugmaier G. The small spleen: sonographic patterns of functional hyposplenia or asplenia. J Clin Ultrasound. 2003;Mar-Apr;31(3):152-5. [Medline].

  10. Harrod VL, Howard TA, Zimmerman SA, Dertinger SD, Ware RE. Quantitative analysis of Howell-Jolly bodies in children with sickle cell disease. Exp Hematol. Feb 2007;35(2):179-83. [Medline].

  11. Katcher AL. Familial asplenia, other malformations, and sudden death. Pediatrics. 1980;65(3):633-635. [Medline].

  12. Kevy SV, Tefft M, Vawier GF, Rosen FS. Hereditary splenic hypoplasia. Pediatrics. Nov 1968;42(5):752-7. [Medline].

  13. Lane PA, O'Connell JL, Lear JL, et al. Functional asplenia in hemoglobin SC disease. Blood. Apr 15 1995;85(8):2238-44. [Medline].

  14. Lin XZ, Chang TM, Tsai HM, et al. Liver, spleen and tumor volume measured by personal computer. Hepatogastroenterology. Mar-Apr 1999;46(26):838-42. [Medline].

  15. Lindor NM, Smithson WA, Ahumada CA, et al. Asplenia in two father-son pairs. Am J Med Genet. Mar 13 1995;56(1):10-1. [Medline].

  16. Long WA. Ivemark syndrome (cardiosplenic or heterotaxy syndrome). In: Fetal and Neonatal Cardiology. 1990:616-19.

  17. Mebius RE, Kraal G. Structure and function of the spleen. Nat Rev Immunol. Aug 2005;5(8):606-16. [Medline].

  18. Melles DC, de Marie S. Prevention of infections in hyposplenic and asplenic patients: an update. Neth J Med. Feb 2004;62(2):45-52. [Medline].

  19. Moller JH, Neal WA. Congenital cardiac anomalies. In: Fetal, Neonatal, and Infant Cardiac Disease. 1990:767-71.

  20. Morgan MS. Prophylaxis should be considered even for trivial animal bites. BMJ. May 10 1997;314(7091):1413. [Medline].

  21. Pearson HA. The spleen and disturbances of splenic function. Hematology. 1993;2:1058-9.

  22. Price VE, Blanchette VS, Ford-Jones EL. The prevention and management of infections in children with asplenia or hyposplenia. Infect Dis Clin North Am. Sep 2007;21(3):697-710, viii-ix. [Medline].

  23. Price VE, Dutta S, Blanchette VS, et al. The prevention and treatment of bacterial infections in children with asplenia or hyposplenia: practice considerations at the Hospital for Sick Children, Toronto. Pediatr Blood Cancer. 2006;May 1;46(5):597-603. [Medline].

  24. Rose V, Izukawa T, Moes CA. Syndromes of asplenia and polysplenia. A review of cardiac and non- cardiac malformations in 60 cases with special reference to diagnosis and prognosis. Br Heart J. Aug 1975;37(8):840-52. [Medline].

  25. Stiehm ER. Immunodeficiency disorders. In: Immunologic Disorders in Infants and Children. 4th ed. 1996:326-327.

  26. Tham KT, Teague MW, Howard CA, Chen SY. A simple splenic reticuloendothelial function test: counting erythrocytes with argyrophilic inclusions. Am J Clin Pathol. May 1996;105(5):548-52. [Medline].

  27. Tribioli C, Lufkin T. The murine Bapx1 homeobox gene plays a critical role in embryonic development of the axial skeleton and spleen. Development. Dec 1999;126(24):5699-711. [Medline].

  28. Waldman JD, Rosenthal A, Smith AL, et al. Sepsis and congenital asplenia. J Pediatr. Apr 1977;90(4):555-9. [Medline].

  29. Wang JK, Hsieh KH. Immunologic study of the asplenia syndrome. Pediatr Infect Dis J. Nov 1991;10(11):819-22. [Medline].

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Further Reading

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Keywords

asplenia, hypoplasia, splenic hypoplasia, absent spleen, nonfunctional spleen, autosplenectomy, hyposplenia, splen, Ivemark syndrome, asplenia syndrome, functional asplenia, congenital asplenia, bacterial sepsis, polysplenia, Klebsiella species, Escherichia coli, Streptococcus pneumoniae, Haemophilus influenzae type b, Neisseria meningitidis, malaria, babesiosis, endocardial cushion defects, pulmonary atresia, pulmonary stenosis, transposition of the great vessels, total anomalous pulmonary venous return

double-outlet right ventricle, atrioventricular canal defects, splenosis, respiratory distress, Pearson syndrome, pancreatic insufficiency, sideroblastic anemia, Stormorken syndrome, thrombocytopenia, miosis, Smith-Fineman-Myers syndrome, mental retardation, short stature, cryptorchidism, ATR-X syndrome, thalassemia, Fanconi anemia, Hodgkin disease, systemic lupus erythematous, SLE, rheumatoid arthritis, inflammatory bowel disease, graft versus host disease, nephrotic syndrome

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Contributor Information and Disclosures

Author

Joseph C Turbyville, MD, Fellow, Department of Allergy and Immunology, Walter Reed Army Medical Center
Joseph C Turbyville, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, and American College of Allergy, Asthma and Immunology
Disclosure: Nothing to disclose.

Coauthor(s)

Cecilia P Mikita, MD, MPH, Associate Program Director, Allergy-Immunology Fellowship, Chief, Clinical Services, Allergy-Immunology Clinic, Walter Reed Army Medical Center
Cecilia P Mikita, MD, MPH is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American College of Allergy, Asthma and Immunology, and Clinical Immunology Society
Disclosure: Nothing to disclose.

Mudra Kumar, MD, MBBS, MRCP, Associate Professor, Department of Pediatrics, University of South Florida College of Medicine
Mudra Kumar, MD, MBBS, MRCP is a member of the following medical societies: American Academy of Pediatrics and American Society of Hematology
Disclosure: Nothing to disclose.

Medical Editor

Ann O'Neill Shigeoka, MD †, Former Clinical Associate Professor, Department of Pediatrics, Division of Immunology-Rheumatology, University of Utah School of Medicine
Ann O'Neill Shigeoka, MD † is a member of the following medical societies: American Federation for Medical Research, Clinical Immunology Society, Pediatric Infectious Diseases Society, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

John Wilson Georgitis, MD, Consulting Staff, Lafayette Allergy Services
John Wilson Georgitis, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association for the Advancement of Science, American College of Chest Physicians, American Lung Association, American Medical Writers Association, and American Thoracic Society
Disclosure: Nothing to disclose.

CME Editor

David Pallares, MD, Clinical Assistant Professor, Department of Pediatrics, Division of Allergy and Immunology, University of Louisville
David Pallares, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology
Disclosure: Nothing to disclose.

Chief Editor

Harumi Jyonouchi, MD, Associate Professor, Division of Pulmonary Allergy/Immunology and Infectious Diseases, Department of Pediatrics, UMDNJ-New Jersey Medical School
Harumi Jyonouchi, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association of Immunologists, American Medical Association, Clinical Immunology Society, New York Academy of Sciences, Society for Experimental Biology and Medicine, Society for Mucosal Immunology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

 
 
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