Hereditary elliptocytosis surgery
|
Hereditary elliptocytosis Microchapters |
|
Differentiating Hereditary elliptocytosis from other Diseases |
|---|
|
Diagnosis |
|
Treatment |
|
Case Studies |
|
Hereditary elliptocytosis surgery On the Web |
|
American Roentgen Ray Society Images of Hereditary elliptocytosis surgery |
|
Risk calculators and risk factors for Hereditary elliptocytosis surgery |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Niyousha Danesh M.D., M.P.H
Overview
Splenectomy may be necessary in a patients with uncompensated hemolysis, presenting clinically significant symptoms. splenectomy remarkably decreases hemolysis in these patients and improves anemia, yet splenectomy can increase the risk of infection so patients should be immunized against Hemophilus influenza, meningococcal and pneumococcal infections before surgery.
Patients with HE have a mild increased risk of developing gallstones. Cholecystitis and cholelithiasis with complications or remaining pain must be treated surgically, through cholecystectomy .[1]
Surgery
Because the spleen is the bodily organ which breaks down old and worn-out blood cells, those individuals with more severe forms of hereditary elliptocytosis can have a splenomegaly which causes a worsening of the signs and symptoms of their anaemia. These can include:
Removal of the spleen (splenectomy) is effective in reducing the severity of these complications, but is associated with an increased risk of overwhelming bacterial septicaemia, and is only performed on those with significant complications. Because many neonates with severe elliptocytosis progress to have only a mild disease, and because this age group is particularly susceptible to pneumococcal infections. Patients with mild hereditary spherocytosis generally do not need splenectomy, it should be done before puberty in moderate forms of HE . Splenectomy is indicated in severe forms,however it is better to be postponed in children until the age of 6. [2]
References
- ↑ Barcellini W, Bianchi P, Fermo E, Imperiali FG, Marcello AP, Vercellati C; et al. (2011). "Hereditary red cell membrane defects: diagnostic and clinical aspects". Blood Transfus. 9 (3): 274–7. doi:10.2450/2011.0086-10. PMC 3136593. PMID 21251470.
- ↑ 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.