eMedicine Specialties > Hematology > Red Blood Cells and Disorders

Spherocytosis, Hereditary: Follow-up

Author: Gus Gonzalez, MD, Medical Oncologist, The Center for Cancer and Blood Disorders
Coauthor(s): E Randy Eichner, MD, Professor, Department of Internal Medicine, University of Oklahoma Health Sciences Center
Contributor Information and Disclosures

Updated: Aug 27, 2009

Follow-up

Further Inpatient Care

  • Evaluation for splenectomy by a surgeon should take place. Consideration of a partial splenectomy can be sought if access to a surgeon experienced in this procedure is possible.

Further Outpatient Care

  • After splenectomy, RBC survival improves dramatically, enabling most patients with HS to maintain a normal hemoglobin level.
  • Ideally, splenectomy should not be performed until a child is older than 6 years because of the increased incidence of postsplenectomy infections with encapsulated organisms such as S pneumoniae and H influenzae in young children.

Inpatient & Outpatient Medications

  • Lifelong folic acid supplementation is recommended for these patients secondary to low levels of chronic hemolysis. This is especially true for those who have not undergone splenectomy.

Complications

  • Often, the patient has had multiple RBC transfusions. This occurs because of multiple hemolytic episodes or an inaccurate diagnosis. Iron overload can become a serious problem, resulting in liver dysfunction. Another cause of iron overload is the addition of oral iron supplementation for the anemia. If a patient presents with mild liver dysfunction, iron store studies should be performed.

Prognosis

  • After splenectomy, RBC survival improves dramatically, enabling most patients with HS to maintain a normal hemoglobin level.

Miscellaneous

Medicolegal Pitfalls

  • Occasionally, patients with HS are incorrectly diagnosed with Gilbert disease.
  • Vaccination against pneumococcal organisms and H influenzae must be administered to patients prior to splenectomy and, indeed, probably to all patients with severe HS.
  • If a partial splenectomy is performed, splenic function is preserved and vaccinations may be delayed until after surgery; however, the long-term data is not well established.

Special Concerns

  • After splenectomy, all patients should have yearly immunizations updated as needed, especially those covering encapsulated organisms.
  • All patients should be placed on supplemental folic acid replacement because of chronic hemolysis.
 


More on Spherocytosis, Hereditary

Overview: Spherocytosis, Hereditary
Differential Diagnoses & Workup: Spherocytosis, Hereditary
Treatment & Medication: Spherocytosis, Hereditary
Follow-up: Spherocytosis, Hereditary
References
Further Reading
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References

  1. Perrotta S, Della Ragione F, Rossi F, et al. {beta}-spectrinBari: a truncated {beta}-chain responsible for dominant hereditary spherocytosis. Haematologica. Jul 16 2009;epub ahead of print. [Medline]. [Full Text].

  2. Maciag M, Plochocka D, Adamowicz-Salach A, Burzynska B. Novel beta-spectrin mutations in hereditary spherocytosis associated with decreased levels of mRNA. Br J Haematol. Aug 2009;146(3):326-32. [Medline].

  3. Perrotta S, Gallagher PG, Mohandas N. Hereditary spherocytosis. Lancet. Oct 18 2008;372(9647):1411-26. [Medline].

  4. Abdullah F, Zhang Y, Camp M, Rossberg MI, Bathurst MA, Colombani PM, et al. Splenectomy in hereditary spherocytosis: Review of 1,657 patients and application of the pediatric quality indicators. Pediatr Blood Cancer. Jul 2009;52(7):834-7. [Medline].

  5. Morinis J, Dutta S, Blanchette V, Butchart S, Langer JC. Laparoscopic partial vs total splenectomy in children with hereditary spherocytosis. J Pediatr Surg. Sep 2008;43(9):1649-52. [Medline].

  6. Grace RF, Mednick RE, Neufeld EJ. Compliance with immunizations in splenectomized individuals with hereditary spherocytosis. Pediatr Blood Cancer. Jul 2009;52(7):865-7. [Medline].

  7. Agre P, Asimos A, Casella JF, McMillan C. Inheritance pattern and clinical response to splenectomy as a reflection of erythrocyte spectrin deficiency in hereditary spherocytosis. N Engl J Med. Dec 18 1986;315(25):1579-83. [Medline].

  8. Costa FF, Agre P, Watkins PC, et al. Linkage of dominant hereditary spherocytosis to the gene for the erythrocyte membrane-skeleton protein ankyrin. N Engl J Med. Oct 11 1990;323(15):1046-50. [Medline].

  9. Gallagher PG. Hematologically important mutations: ankyrin variants in hereditary spherocytosis. Blood Cells Mol Dis. Nov-Dec 2005;35(3):345-7.

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  12. Hassoun H, Vassiliadis JN, Murray J, et al. Hereditary spherocytosis with spectrin deficiency due to an unstable truncated beta spectrin. Blood. Mar 15 1996;87(6):2538-45. [Medline].

  13. Korones D, Pearson HA. Normal erythrocyte osmotic fragility in hereditary spherocytosis. J Pediatr. Feb 1989;114(2):264-6. [Medline].

  14. Lee RD, Glader JN, Lukens JN. In: Lee GR, Foerster J, Lukens J, Paraskevas F, Greer JP, Rodgers GM, eds. Wintrobe's Clinical Hematology. 10th ed. Baltimore, Md: Lippincott Williams & Wilkins; 1999:. 1132-42.

  15. Nakashima K, Beutler E. Erythrocyte cellular and membrane deformability in hereditary spherocytosis. Blood. Mar 1979;53(3):481-5. [Medline].

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  17. Rice HE, Oldham KT, Hillery CA, et al. Clinical and hematologic benefits of partial splenectomy for congenital hemolytic anemias in children. Ann Surg. Feb 2003;237(2):281-8.

  18. Sackey K. Hemolytic anemia: Part 1. Pediatr Rev. May 1999;20(5):152-8; quiz 159. [Medline].

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  21. Stoehr GA, Stauffer UG, Eber SW. Near-total splenectomy: a new technique for the management of hereditary spherocytosis. Ann Surg. Jan 2005;241(1):40-7.

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Further Reading

Related eMedicine Topics

Clinical Trials

National Guideline Clearinghouse

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Keywords

hereditary spherocytosis, HS, congenital spherocytosis, familial hemolytic disorder, hemolytic anemia, anemia, blood disorder, hereditary hemolytic anemia, aplastic crisis, megaloblastic crisis, hemolytic crisis, cholecystitis, cholelithiasis, neonatal hemolysis, splenomegaly

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Contributor Information and Disclosures

Author

Gus Gonzalez, MD, Medical Oncologist, The Center for Cancer and Blood Disorders
Gus Gonzalez, MD is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine
Disclosure: Nothing to disclose.

Coauthor(s)

E Randy Eichner, MD, Professor, Department of Internal Medicine, University of Oklahoma Health Sciences Center
E Randy Eichner, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Sports Medicine, American Medical Society for Sports Medicine, and American Society of Hematology
Disclosure: Nothing to disclose.

Medical Editor

Paul Schick, MD, Emeritus Professor, Department of Internal Medicine, Thomas Jefferson University Medical College; Research Professor, Department of Internal Medicine, Drexel University College of Medicine; Adjunct Professor of Medicine, Lankenau Hospital, Wynnewood, PA
Paul Schick, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Society of Hematology, International Society on Thrombosis and Haemostasis, and New York Academy of Sciences
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Ronald A Sacher, MB, BCh, MD, FRCPC, Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center
Ronald A Sacher, MB, BCh, MD, FRCPC is a member of the following medical societies: American Society of Hematology
Disclosure: Glaxo Smith Kline Honoraria Speaking and teaching; Talecris Honoraria Board membership

CME Editor

Rajalaxmi McKenna, MD, FACP, Consulting Staff, Department of Medicine, Southwest Medical Consultants, SC, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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