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{{Hereditary spherocytosis}} | {{Hereditary spherocytosis}} | ||
{{CMG}} {{AE}} | {{CMG}} {{AE}} | ||
==Overview== | ==Overview== | ||
Generally, the treatment of [[hereditary spherocytosis]] involves presplenectomy care, [[splenectomy]] and management of postsplenectomy [[Complication (medicine)|complications]]. [[Splenectomy]] is very effective in reducing [[hemolysis]], leading to significant prolongation of [[red blood cell]] [[lifespan]]. Partial [[Splenectomy|splenectomies]] can be used in [[Pediatrics|pediatric]] [[Patient|patients]] as it controls [[hemolysis]] and preserves [[Spleen|splenic]] [[Function (biology)|function]]. [[Patient|Patients]] having concomitant [[Gallstone disease|gallstones]] are likely to benefit from combined [[splenectomy]] and [[cholecystectomy]] in terms of [[life expectancy]]. Post [[splenectomy]] [[Complication (medicine)|complications]] may include; [[Infection|infections]] & [[sepsis]] caused by [[encapsulated organisms]] ([[streptococcus pneumoniae]], [[neisseria meningitidis]], [[Haemophilus influenzae|haemophilus influenza]]), [[Deep vein thrombosis|deep venous thrombosis (DVT)]], [[Pulmonary embolism|pulmonary emboli]] and [[portal vein thrombosis]]. | |||
==Surgery== | ==Surgery== | ||
=== Splenectomy: === | * '''Splenectomy''' | ||
* Generally, the treatment of | ** [[Splenectomy]] is very effective in reducing [[hemolysis]], leading to significant prolongation of the [[red blood cell]] [[lifespan]].<ref>{{Cite journal | ||
* In pediatric cases, splenectomy ideally 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 | | author = [[P. H. B. Bolton-Maggs]], [[R. F. Stevens]], [[N. J. Dodd]], [[G. Lamont]], [[P. Tittensor]] & [[M.-J. King]] | ||
* Partial splenectomies are increasingly used in pediatric patients, as this approach appears to both control hemolysis and preserve splenic function. | | title = Guidelines for the diagnosis and management of hereditary spherocytosis | ||
* European guidelines on splenectomy for | | journal = [[British journal of haematology]] | ||
* | | volume = 126 | ||
| issue = 4 | |||
| pages = 455–474 | |||
| year = 2004 | |||
| month = August | |||
| doi = 10.1111/j.1365-2141.2004.05052.x | |||
| pmid = 15287938 | |||
}}</ref><ref name="CasalePerrotta2014">{{cite journal|last1=Casale|first1=Maddalena|last2=Perrotta|first2=Silverio|title=Splenectomy for hereditary spherocytosis: complete, partial or not at all?|journal=Expert Review of Hematology|volume=4|issue=6|year=2014|pages=627–635|issn=1747-4086|doi=10.1586/ehm.11.51}}</ref> | |||
** [[Patient|Patients]] should be selected for [[splenectomy]] on the basis of their [[clinical]] [[Symptom|symptoms]] and presence of [[Complication (medicine)|complications]] such as [[Gallstone disease|gallstones]], not simply on the basis of [[diagnosis]] alone. | |||
** Generally, the treatment of [[hereditary spherocytosis]] involves presplenectomy care, [[splenectomy]], and management of postsplenectomy [[Complication (medicine)|complications]]. | |||
** In [[Pediatrics|pediatric]] cases, [[splenectomy]] ideally should not be performed until a [[child]] is older than 6 years because of the increased [[incidence]] of postsplenectomy [[Infection|infections]] with [[encapsulated organisms]] such as [[streptococcus pneumoniae]] and [[haemophilus influenzae]] in young [[Child|children]]. | |||
** Partial splenectomies are increasingly used in [[Pediatrics|pediatric]] [[Patient|patients]], as this approach appears to both control [[hemolysis]] and preserve [[Spleen|splenic]] [[Function (biology)|function]]. | |||
** European guidelines on [[splenectomy]] for [[hereditary spherocytosis]] note that a [[Laparoscopic surgery|laparoscopic]] approach is currently considered the gold standard for removal of a normal-sized or slightly enlarged [[spleen]] and is preferred to open [[splenectomy]], but it should be performed only by experienced [[Surgeon|surgeons]]. | |||
** [[Child|Children]] or young [[Adult|adults]] with mild [[hereditary spherocytosis]] who also has [[Gallstone disease|gallstones]] are likely to benefit from combined [[splenectomy]] and [[cholecystectomy]] in terms of [[life expectancy]].<ref name="Bolton-MaggsStevens2004">{{cite journal|last1=Bolton-Maggs|first1=P. H. B.|last2=Stevens|first2=R. F.|last3=Dodd|first3=N. J.|last4=Lamont|first4=G.|last5=Tittensor|first5=P.|last6=King|first6=M.-J.|title=Guidelines for the diagnosis and management of hereditary spherocytosis|journal=British Journal of Haematology|volume=126|issue=4|year=2004|pages=455–474|issn=0007-1048|doi=10.1111/j.1365-2141.2004.05052.x}}</ref> | |||
** Following [[splenectomy]], the [[clinical]] manifestations and [[Complication (medicine)|complications]] ([[anemia]] & [[Gallstone disease|gallstones]]) are much reduced in severe [[hereditary spherocytosis]] and abolished in milder cases, but at the risk of increased life threatening [[sepsis]] from [[encapsulated organisms]], particularly [[streptococcus pneumoniae]].<ref name="Bolton-MaggsStevens2004">{{cite journal|last1=Bolton-Maggs|first1=P. H. B.|last2=Stevens|first2=R. F.|last3=Dodd|first3=N. J.|last4=Lamont|first4=G.|last5=Tittensor|first5=P.|last6=King|first6=M.-J.|title=Guidelines for the diagnosis and management of hereditary spherocytosis|journal=British Journal of Haematology|volume=126|issue=4|year=2004|pages=455–474|issn=0007-1048|doi=10.1111/j.1365-2141.2004.05052.x}}</ref><ref>{{Cite journal | |||
| author = [[Sayeeda Huq]], [[Mark A. C. Pietroni]], [[Hafizur Rahman]] & [[Mohammad Tariqul Alam]] | |||
| title = Hereditary spherocytosis | |||
| journal = [[Journal of health, population, and nutrition]] | |||
| volume = 28 | |||
| issue = 1 | |||
| pages = 107–109 | |||
| year = 2010 | |||
| month = February | |||
| pmid = 20214092 | |||
}}</ref> | |||
* '''Complications of splenectomy''' | |||
** [[Splenectomy]] has a number of known risks of which [[Patient|patients]] (or parents) should be aware<ref name="pmid28550188">{{cite journal| author=Iolascon A, Andolfo I, Barcellini W, Corcione F, Garçon L, De Franceschi L et al.| title=Recommendations regarding splenectomy in hereditary hemolytic anemias. | journal=Haematologica | year= 2017 | volume= 102 | issue= 8 | pages= 1304-1313 | pmid=28550188 | doi=10.3324/haematol.2016.161166 | pmc=5541865 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28550188 }}</ref> | |||
** Operative risks (eg, [[infection]], [[bleeding]], or [[injury]] to adjacent [[Organ (anatomy)|organs]] such as the [[stomach]] or [[Tail of pancreas|tail of the pancreas]]); these are relatively infrequent. | |||
** [[Infection|Infections]], including overwhelming [[sepsis]], from [[encapsulated organisms]] ([[streptococcus pneumoniae]], [[neisseria meningitidis]], [[Haemophilus influenzae|hemophilus influenzae]]) that can no longer be removed by normal [[Spleen|splenic]] clearance mechanisms, as well as certain other [[Microorganism|microorganisms]] including [[Plasmodium|plasmodia]], [[Babesia]], [[Bordetella]] and [[Capnocytophaga]] [[species]] (from [[Animal bite|animal bites]]).<ref name="pmid285501882">{{cite journal| author=Iolascon A, Andolfo I, Barcellini W, Corcione F, Garçon L, De Franceschi L et al.| title=Recommendations regarding splenectomy in hereditary hemolytic anemias. | journal=Haematologica | year= 2017 | volume= 102 | issue= 8 | pages= 1304-1313 | pmid=28550188 | doi=10.3324/haematol.2016.161166 | pmc=5541865 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28550188 }}</ref> | |||
** These risks are thought to be highest in the [[Infant|first year]] following [[splenectomy]] and in individuals undergoing [[splenectomy]] before five to six years of [[Ageing|age]]. | |||
** However, risks of [[sepsis]] are likely to have declined with improved options for preoperative [[Vaccination|vaccinations]] and postoperative [[Prophylaxis|prophylactic]] [[penicillin]]. This was illustrated in a 1991 study from the Danish National Patient Registry that demonstrated a dramatic reduction in serious [[Streptococcus pneumoniae|streptococcus pneumoniae infections]] following [[Pneumococcal vaccine|pneumococcal vaccination]].<ref name="pmid2058391">{{cite journal| author=Konradsen HB, Henrichsen J| title=Pneumococcal infections in splenectomized children are preventable. | journal=Acta Paediatr Scand | year= 1991 | volume= 80 | issue= 4 | pages= 423-7 | pmid=2058391 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2058391 }}</ref> | |||
** Individuals who did not receive appropriate pre-splenectomy [[Vaccination|vaccinations]] should have a thorough review of their [[immunization]] history and should receive [[Vaccination|vaccinations]]. | |||
** [[Venous thromboembolism|Venous thromboembolic (VTE)]] [[Complication (medicine)|complications]] including [[Deep vein thrombosis|thromboses of the deep veins]], [[Pulmonary embolism|pulmonary emboli]], [[Splenic vein thrombosis|splenic]] or [[portal vein thrombosis]], as well as [[thrombosis]] in other unusual sites.<ref name="pmid27118929">{{cite journal| author=Davidsen C, Larsen TH, Gerdts E, Lønnebakken MT| title=Giant right ventricular outflow tract thrombus in hereditary spherocytosis: a case report. | journal=Thromb J | year= 2016 | volume= 14 | issue= | pages= 9 | pmid=27118929 | doi=10.1186/s12959-016-0083-3 | pmc=4845368 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27118929 }}</ref><ref name="pmid19373154">{{cite journal| author=Perkins LA, Jones SF, Bhargava RS| title=Dural venous thrombosis following splenectomy in a patient with hereditary spherocytosis. | journal=South Med J | year= 2009 | volume= 102 | issue= 5 | pages= 542-5 | pmid=19373154 | doi=10.1097/SMJ.0b013e31819e90b5 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19373154 }}</ref> | |||
** [[Venous thromboembolism]] events appear to be more common in individuals with [[hereditary spherocytosis]] who undergo [[splenectomy]] than in those who do not, but the individuals who undergo [[splenectomy]] may have had more severe underlying [[disease]], making direct comparisons difficult. | |||
** [[Prophylaxis|Thromboprophylaxis]] at the time of [[surgery]] should be used based on standard practices; there is no indication for extended [[Prophylaxis|thromboprophylaxis]] beyond the usual duration. | |||
** [[Arterial thrombosis|Arterial thrombotic events]] may also be increased relative to individuals with [[hereditary spherocytosis]] who do not undergo [[splenectomy]], with the same caveat that applies to [[venous thromboembolism]] (patients who undergo [[splenectomy]] may have more severe underlying [[disease]]).<ref name="pmid18485083">{{cite journal| author=Schilling RF, Gangnon RE, Traver MI| title=Delayed adverse vascular events after splenectomy in hereditary spherocytosis. | journal=J Thromb Haemost | year= 2008 | volume= 6 | issue= 8 | pages= 1289-95 | pmid=18485083 | doi=10.1111/j.1538-7836.2008.03024.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18485083 }}</ref><ref name="pmid9400518">{{cite journal| author=Schilling RF| title=Spherocytosis, splenectomy, strokes, and heat attacks. | journal=Lancet | year= 1997 | volume= 350 | issue= 9092 | pages= 1677-8 | pmid=9400518 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9400518 }}</ref> | |||
** It is not clear whether [[Pulmonary artery hypertension|pulmonary artery hypertension (PAH)]] is a [[Complication (medicine)|complication]] of [[splenectomy]] in [[hereditary spherocytosis]].<ref name="pmid17497044">{{cite journal| author=Smedema JP, Louw VJ| title=Pulmonary arterial hypertension after splenectomy for hereditary spherocytosis. | journal=Cardiovasc J Afr | year= 2007 | volume= 18 | issue= 2 | pages= 84-9 | pmid=17497044 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17497044 }}</ref> | |||
==References== | ==References== | ||
{{reflist|2}} | {{reflist|2}} | ||
Latest revision as of 03:59, 19 December 2018
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:
Overview
Generally, the treatment of hereditary spherocytosis involves presplenectomy care, splenectomy and management of postsplenectomy complications. Splenectomy is very effective in reducing hemolysis, leading to significant prolongation of red blood cell lifespan. Partial splenectomies can be used in pediatric patients as it controls hemolysis and preserves splenic function. Patients having concomitant gallstones are likely to benefit from combined splenectomy and cholecystectomy in terms of life expectancy. Post splenectomy complications may include; infections & sepsis caused by encapsulated organisms (streptococcus pneumoniae, neisseria meningitidis, haemophilus influenza), deep venous thrombosis (DVT), pulmonary emboli and portal vein thrombosis.
Surgery
- Splenectomy
- Splenectomy is very effective in reducing hemolysis, leading to significant prolongation of the red blood cell lifespan.[1][2]
- Patients should be selected for splenectomy on the basis of their clinical symptoms and presence of complications such as gallstones, not simply on the basis of diagnosis alone.
- Generally, the treatment of hereditary spherocytosis involves presplenectomy care, splenectomy, and management of postsplenectomy complications.
- In pediatric cases, splenectomy ideally 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 streptococcus pneumoniae and haemophilus influenzae in young children.
- Partial splenectomies are increasingly used in pediatric patients, as this approach appears to both control hemolysis and preserve splenic function.
- European guidelines on splenectomy for hereditary spherocytosis note that a laparoscopic approach is currently considered the gold standard for removal of a normal-sized or slightly enlarged spleen and is preferred to open splenectomy, but it should be performed only by experienced surgeons.
- Children or young adults with mild hereditary spherocytosis who also has gallstones are likely to benefit from combined splenectomy and cholecystectomy in terms of life expectancy.[3]
- Following splenectomy, the clinical manifestations and complications (anemia & gallstones) are much reduced in severe hereditary spherocytosis and abolished in milder cases, but at the risk of increased life threatening sepsis from encapsulated organisms, particularly streptococcus pneumoniae.[3][4]
- Complications of splenectomy
- Splenectomy has a number of known risks of which patients (or parents) should be aware[5]
- Operative risks (eg, infection, bleeding, or injury to adjacent organs such as the stomach or tail of the pancreas); these are relatively infrequent.
- Infections, including overwhelming sepsis, from encapsulated organisms (streptococcus pneumoniae, neisseria meningitidis, hemophilus influenzae) that can no longer be removed by normal splenic clearance mechanisms, as well as certain other microorganisms including plasmodia, Babesia, Bordetella and Capnocytophaga species (from animal bites).[6]
- These risks are thought to be highest in the first year following splenectomy and in individuals undergoing splenectomy before five to six years of age.
- However, risks of sepsis are likely to have declined with improved options for preoperative vaccinations and postoperative prophylactic penicillin. This was illustrated in a 1991 study from the Danish National Patient Registry that demonstrated a dramatic reduction in serious streptococcus pneumoniae infections following pneumococcal vaccination.[7]
- Individuals who did not receive appropriate pre-splenectomy vaccinations should have a thorough review of their immunization history and should receive vaccinations.
- Venous thromboembolic (VTE) complications including thromboses of the deep veins, pulmonary emboli, splenic or portal vein thrombosis, as well as thrombosis in other unusual sites.[8][9]
- Venous thromboembolism events appear to be more common in individuals with hereditary spherocytosis who undergo splenectomy than in those who do not, but the individuals who undergo splenectomy may have had more severe underlying disease, making direct comparisons difficult.
- Thromboprophylaxis at the time of surgery should be used based on standard practices; there is no indication for extended thromboprophylaxis beyond the usual duration.
- Arterial thrombotic events may also be increased relative to individuals with hereditary spherocytosis who do not undergo splenectomy, with the same caveat that applies to venous thromboembolism (patients who undergo splenectomy may have more severe underlying disease).[10][11]
- It is not clear whether pulmonary artery hypertension (PAH) is a complication of splenectomy in hereditary spherocytosis.[12]
References
- ↑ P. H. B. Bolton-Maggs, R. F. Stevens, N. J. Dodd, G. Lamont, P. Tittensor & M.-J. King (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. PMID 15287938. Unknown parameter
|month=ignored (help) - ↑ Casale, Maddalena; Perrotta, Silverio (2014). "Splenectomy for hereditary spherocytosis: complete, partial or not at all?". Expert Review of Hematology. 4 (6): 627–635. doi:10.1586/ehm.11.51. ISSN 1747-4086.
- ↑ 3.0 3.1 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.
- ↑ Sayeeda Huq, Mark A. C. Pietroni, Hafizur Rahman & Mohammad Tariqul Alam (2010). "Hereditary spherocytosis". Journal of health, population, and nutrition. 28 (1): 107–109. PMID 20214092. Unknown parameter
|month=ignored (help) - ↑ Iolascon A, Andolfo I, Barcellini W, Corcione F, Garçon L, De Franceschi L; et al. (2017). "Recommendations regarding splenectomy in hereditary hemolytic anemias". Haematologica. 102 (8): 1304–1313. doi:10.3324/haematol.2016.161166. PMC 5541865. PMID 28550188.
- ↑ Iolascon A, Andolfo I, Barcellini W, Corcione F, Garçon L, De Franceschi L; et al. (2017). "Recommendations regarding splenectomy in hereditary hemolytic anemias". Haematologica. 102 (8): 1304–1313. doi:10.3324/haematol.2016.161166. PMC 5541865. PMID 28550188.
- ↑ Konradsen HB, Henrichsen J (1991). "Pneumococcal infections in splenectomized children are preventable". Acta Paediatr Scand. 80 (4): 423–7. PMID 2058391.
- ↑ Davidsen C, Larsen TH, Gerdts E, Lønnebakken MT (2016). "Giant right ventricular outflow tract thrombus in hereditary spherocytosis: a case report". Thromb J. 14: 9. doi:10.1186/s12959-016-0083-3. PMC 4845368. PMID 27118929.
- ↑ Perkins LA, Jones SF, Bhargava RS (2009). "Dural venous thrombosis following splenectomy in a patient with hereditary spherocytosis". South Med J. 102 (5): 542–5. doi:10.1097/SMJ.0b013e31819e90b5. PMID 19373154.
- ↑ Schilling RF, Gangnon RE, Traver MI (2008). "Delayed adverse vascular events after splenectomy in hereditary spherocytosis". J Thromb Haemost. 6 (8): 1289–95. doi:10.1111/j.1538-7836.2008.03024.x. PMID 18485083.
- ↑ Schilling RF (1997). "Spherocytosis, splenectomy, strokes, and heat attacks". Lancet. 350 (9092): 1677–8. PMID 9400518.
- ↑ Smedema JP, Louw VJ (2007). "Pulmonary arterial hypertension after splenectomy for hereditary spherocytosis". Cardiovasc J Afr. 18 (2): 84–9. PMID 17497044.