Twenty Years of Splenectomy for Hereditary Spherocytosis

Rutkow, Ira M.

doi:10.1001/archsurg.1981.01380150034008

Cited by 23 publications

(8 citation statements)

References 8 publications

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“…Symptoms of cholelithiasis remain a prime reason for carrying out a splenectomy in hereditary spherocytosis. Stones themselves are reported to be present in 21–63% of cases (Rutkow, 1981). Once the spleen is removed, individuals with HS do not develop pigment stones.…”

Section: Should a Concurrent Cholecystectomy Be Performed?mentioning

confidence: 99%

Guidelines for the diagnosis and management of hereditary spherocytosis

et al. 2004

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SummaryHereditary spherocytosis (HS) is a heterogeneous group of disorders with regard to clinical severity, protein defects and mode of inheritance. It is relatively common in Caucasian populations; most affected individuals have mild or only moderate haemolysis. There is usually a family history, and a typical clinical and laboratory picture so that the diagnosis is often easily made without additional laboratory tests. Atypical cases may require measurement of erythrocyte membrane proteins to clarify the nature of the membrane disorder and in the absence of a family history, occasionally molecular genetic analysis will help to determine whether inheritance is recessive or non-dominant. It is particularly important to rule out stomatocytosis where splenectomy is contraindicated because of the thrombotic risk. Mild HS can be managed without folate supplements and does not require splenectomy. Moderately and severely affected individuals are likely to benefit from splenectomy, which should be performed after the age of 6 years and with appropriate counselling about the infection risk. In all cases careful dialogue between doctor, patient and the family is essential. Laparoscopic surgery, when performed by experienced surgeons, can result in a shorter hospital stay and less pain.Keywords: spherocytosis, hereditary, splenectomy, child, erythrocyte membrane.Hereditary spherocytosis (HS) was described in 1871 and the first recorded splenectomy was performed soon after. It is the commonest cause of inherited chronic haemolysis in Northern Europe and North America with a quoted incidence of 1 in 5000 births (Morton et al, 1962). However, studies of osmotic fragility in blood donors suggest the existence of extremely mild or subclinical forms (Godal & Heisto, 1981;Eber et al, 1992), raising the prevalence of HS to 1 in 2000 . HS has also been found in other ethnic groups (in Africa, Algeria, Tunisia, Egypt, Japan, North India and Brazil). There are only rare cases reported in the black population. In the last 15 years significant progress has been made in the understanding of the biochemical and molecular genetic basis of HS. The definitions of the types of evidence and the grading recommendations used in this guideline are listed in Appendix. Search strategyIn addition to personal archives and consultation of standard textbooks (together with relevant cited references therein), the writing group searched Medline and Embase to identify relevant literature including meta-analyses (none found), reviews and original papers in any language, using the following key words and combinations of them: hereditary spherocytosis; red cell membrane; spectrin, ankyrin, band 3, spherocytes; haemolysis; folate; folic acid; splenectomy; splenectomy in haematological

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Section: Should a Concurrent Cholecystectomy Be Performed?mentioning

confidence: 99%

Guidelines for the diagnosis and management of hereditary spherocytosis

et al. 2004

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“…Stones are reported to be present in 21–63% of patients with HS (Rutkow, 1981). Symptoms of cholelithiasis remain a prime reason for carrying out a splenectomy in HS.…”

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confidence: 99%

Guidelines for the diagnosis and management of hereditary spherocytosis – 2011 update

Bolton‐Maggs¹,

Langer²,

Iolascon³

et al. 2011

Br J Haematol

309

304

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SummaryGuidelines on hereditary spherocytosis (HS) published in 2004 (Bolton-Maggs et al, 2004) are here replaced to reflect changes in current opinion on the surgical management, (particularly the indications for concomitant splenectomy with cholecystectomy in children with mild HS, and concomitant cholecystectomy with splenectomy in those with asymptomatic gallstones).Further potential long term hazards of splenectomy are now recognised. Advances have been made in our understanding of the biochemistry of the red cell membrane which underpins the choice of tests. Biochemical assays of membranes proteins and genetic analysis may be indicated (rarely) to diagnose atypical cases. The diagnostic value of the eosin-5-maleimide (EMA) binding test has been validated in a number of studies with understanding of its limitations.Keywords: spherocytosis, hereditary, splenectomy, child, erythrocyte membrane.The guideline group was selected to represent UK medical experts and patient representatives but sought the expertise of two overseas specialists with a particular interest in hereditary spherocytosis (HS).The writing group searched PubMed from 2003 to July 2010 for relevant literature including meta-analyses (none found), reviews and original papers in any language, using the following key words and combinations of them: hereditary spherocytosis; red cell membrane; spectrin; ankyrin; band 3; spherocytes; haemolysis; folate; folic acid; splenectomy; cholecystectomy; cholecystostomy; laparoscopic; gallstones; pneumococcal; vaccination; penicillin prophylaxis. Only the abstracts were read of papers in languages other than English. The writing group produced the draft guideline, which was subsequently revised by consensus by members of the General Haematology Task Force of the British Committee for Standards in Haematology (BCSH). The guideline was then reviewed by a sounding board of approximately 50 UK haematologists, the BSCH and the British Society for Haematology Committee and comments incorporated where appropriate. The 'GRADE' system was used to quote levels and grades of evidence, details of which can be found in Appendix I. The objective of this guideline is to provide healthcare professionals with clear guidance on the management of HS. In all cases individual patient circumstances may dictate an alternative approach. Summary of key recommendationsDiagnostic testing (confirmation of previous guidelines)

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“…The presence ofthe spleen is essential for the membrane defeet to express itself as a haemolytic process. For this reason splenectomy is indicated (Lennert 1972, Rutkow 1981. The haemoglobin value in the HA series was not significantly raised during the first postoperative weeks.…”

Section: Resultsmentioning

confidence: 98%

“…The principle features of this process include increased rate of red blood cell destruction, spherocytosis, splenomegaly and familial occurrence. The excessive red blood cell destruction can result elinically in anaemia, reticulocytosis, increased serum iron eoncentration, achol uric jaundice, skeletal anomalies and biliary tract disease in the form of cholelithiasis (Rutkow 1981). The anaemia may be severe and become apparent in early childhood or the disease may be detected in adulthood.…”

Section: Haemolytic Anaemiamentioning

confidence: 99%

Splenectomy in Children with Haematological Disease

Cm¹,

Garwicz²

1982

Eur J Pediatr Surg

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During the years 1969-1980 elective splenectomy for haematological disease was performed in 31 children, of which 17 had haemolytic anaemia (HA), 11 thrombocytopenic purpura (TP) and 3 hypersplenism (HS). The indications for splenectomy in the HA series were haemolytic crises in 6 children, constant anaemia in 4, hyperbilirubinaemia in 5 and abdominal pain in 2 children. Among children with TP there were 8 cases of idiopathic thrombocytopenic purpura, 2 cases combined TP with haemolytic anaemia and idiopathic pancytopenia in 1 child. In all patients the indication for splenectomy was therapy-resistant thrombocytopenia. In the HS series the underlying disease was Morbus Gaucher, thyrosinosis and cysta lienalis. Indication for splenectomy was thrombocytopenia in all children. In the HA series the results of splenectomy were good in all patients. The haemoglobin value rose. The increased reticulocyte count returned to normal values. Splenectomy was considered to be still indicated in all patients with hereditary spherocytosis. In the TP series good results were obtained in 6 children, 2 were markedly improved, but 3 showed no change. Splenectomy plays an important role in the management of chronic thrombocytopenia when medical treatment is without effect. In the HS series thrombocytes reached normal values in all patients. Failure of splenectomy to relieve the haematologic process is usually due to remaining accessory spleens. Splenectomy in children increases the risk of severe infections and the polyvalent pneumococcal vaccine should therefore be administered prior to splenectomy.

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Twenty Years of Splenectomy for Hereditary Spherocytosis

Cited by 23 publications

References 8 publications

Guidelines for the diagnosis and management of hereditary spherocytosis

Guidelines for the diagnosis and management of hereditary spherocytosis

Guidelines for the diagnosis and management of hereditary spherocytosis – 2011 update

Splenectomy in Children with Haematological Disease

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