Hereditary spherocytosis medical therapy

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Hereditary spherocytosis Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

There is no specific medical therapy for the hereditary spherocytosis, as the diagnosis is made, surveillance is needed to help detect and manage any complications. A routine annual review is usually sufficient to detect any complications. Folic acid supplementation is not always required, but is used as a routine for children with severe hemolysis and in pregnancy regardless of severity of disease. Blood transfusion may also be required in severely affected infants and may be needed during aplastic crisis or pregnancy. However, erythropoietin (EPO) may be helpful in reducing the need for transfusion in some infants.

Medical Therapy

References

  1. Bolton-Maggs, P. H. B.; Stevens, R. F.; Dodd, N. J.; Lamont, G.; Tittensor, P.; King, M.-J. (2004). "Guidelines for the diagnosis and management of hereditary spherocytosis". British Journal of Haematology. 126 (4): 455–474. doi:10.1111/j.1365-2141.2004.05052.x. ISSN 0007-1048.
  2. P. H. B. Bolton-Maggs (2004). "Hereditary spherocytosis; new guidelines". Archives of disease in childhood. 89 (9): 809–812. doi:10.1136/adc.2003.034587. PMID 15321852. Unknown parameter |month= ignored (help)
  3. Bolton-Maggs PH, Stevens RF, Dodd NJ, Lamont G, Tittensor P, King MJ; et al. (2004). "Guidelines for the diagnosis and management of hereditary spherocytosis". Br J Haematol. 126 (4): 455–74. doi:10.1111/j.1365-2141.2004.05052.x. PMID 15287938.
  4. Bolton-Maggs PH, Langer JC, Iolascon A, Tittensor P, King MJ, General Haematology Task Force of the British Committee for Standards in Haematology (2012). "Guidelines for the diagnosis and management of hereditary spherocytosis--2011 update". Br J Haematol. 156 (1): 37–49. doi:10.1111/j.1365-2141.2011.08921.x. PMID 22055020.
  5. Tchernia G, Delhommeau F, Perrotta S, Cynober T, Bader-Meunier B, Nobili B; et al. (2000). "Recombinant erythropoietin therapy as an alternative to blood transfusions in infants with hereditary spherocytosis". Hematol J. 1 (3): 146–52. doi:10.1038/sj/thj/6200022. PMID 11920183.
  6. Zhang XH, Fu HX, Xu LP, Liu DH, Chen H, Han W; et al. (2012). "Allo-hematopoietic stem cell transplantation is a potential treatment for a patient with a combined disorder of hereditary spherocytosis". Chin Med J (Engl). 125 (5): 947–50. PMID 22490603.
  7. Tchernia G, Delhommeau F, Perrotta S, Cynober T, Bader-Meunier B, Nobili B; et al. (2000). "Recombinant erythropoietin therapy as an alternative to blood transfusions in infants with hereditary spherocytosis". Hematol J. 1 (3): 146–52. doi:10.1038/sj/thj/6200022. PMID 11920183.

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