Clinical and Hematologic Benefits of Partial Splenectomy for Congenital Hemolytic Anemias in Children

Rice, Henry E.; Oldham, Keith T.; Hillery, Cheryl A.; Skinner, Michael A.; O’Hara, Sara M.; Ware, Russell E.

doi:10.1097/01.sla.0000048453.61168.8f

Cited by 88 publications

(91 citation statements)

References 25 publications

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“…(26,29) Vasilescu et al (24,30) have shown that preservation of the lower pole of the spleen allows a better mobilization of the splenic remnant, and a more accurate assessment of the volume. The length of the splenic remnant ranged between 3.5 and 6 cm, depending on the initial size of the spleen.…”

Section: The Role Of Splenectomy In Hereditary Spherocytosismentioning

confidence: 99%

“…In children less than 5 years old, the risk of overwhelming postsplenectomy sepsis may be increased 60-to 100 fold, compared with that of children without a splenectomy (18) There are also concerns that despite the immunizations against encapsulated bacteria and the postoperative antibiotic prophylaxis, the risk after total splenectomy is never eliminated. (17,19,20) Because of this reason, a number of alternatives to total splenectomy have been considered, including auto-transplantation of the removed spleen and partial splenectomy. (21) The use of partial splenectomy, particularly in pediatric patients, aims to retain splenic immunologic function, while at the same time to decrease the rate of hemolysis.…”

Section: The Role Of Splenectomy In Hereditary Spherocytosismentioning

confidence: 99%

“…(22)(23)(24) Several studies have set the minimal amount of the residual parenchyma necessary to maintain adequate phagocytic function, to approximately 15% of the enlarged spleen. (25)(26)(27) Technically, a splenectomy can be performed either in the traditional open manner or with a minimally invasive approach.…”

Section: The Role Of Splenectomy In Hereditary Spherocytosismentioning

confidence: 99%

See 2 more Smart Citations

Hereditary Spherocytosis - Diagnosis, Surgical Treatment and Outcomes. A Literature Review

Manciu¹,

Matei²,

Trandafir³

2017

chr

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RezumatSferocitoza ereditarã este o maladie congenitalã, caracterizatã prin anemie hemoliticã, ce afecteazã membranele eritrocitelor. Manifestãrile clinice variazã de la forme aproape asimptomatice la forme severe, ce necesitã transfuzii. Diagnosticul se bazeazã pe examenul clinic, hemograma cu numãrul reticulocitelor, prezenţa unui istoric familial şi teste de laborator specific, cum ar fi testul EMA (eosin-5-maleimide binding test) sau testul AGLT (Acidified Glycerol Lysis Time). Splenectomia este consideratã tratamentul standard în formele moderate pânã la severe de boalã. Totuşi, este cunoscut faptul cã splenectomia totalã expune pacientul la complicaţii infecţioase, uneori letale, ceea ce a determinat indicarea acestei metode cu precauţie. Astfel splenectomia subtotalã sau parţialã a devenit o alternativã de tratament fezabilã. Aceasta reduce distrugerea eritrocitelor, în timp ce conservã funcţia imunã a splinei. Majoritatea studiilor au arãtat beneficii certe, pe termen scurt, dupã splenectomie, în cazul acestor pacienţi. Totuşi, pânã în prezent, rezultate pe termen lung certe, adicã pe o perioadã de peste 5 ani de urmãrire postoperatorie, încã lipsesc.Cuvinte cheie: sferocite, hemoliză, citometrie în flux, splenectomie, rezultate pe termen lung AbstractHereditary spherocytosis (HS) is a disease affecting the red blood cells membrane and belongs to the congenital hemolytic anemias. The clinical spectrum ranges from asymptomatic patients to severe forms requiring transfusions in early childhood. The diagnosis can be based on the physical examination, complete red blood cell count, reticulocytes count, medical history and specific tests, preferentially the EMA test (eosin-5-maleimide binding) test and AGLT (Acidified Glycerol Lysis Time). Splenectomy is considered the standard surgical treatment in moderate and severe forms of hereditary spherocytosis. Total splenectomy exposes the patient to a life -long risk of potentially lethal infections and thus, its usage was reconsidered. Because of this reason, a feasible alternative is the partial splenectomy. The use of partial splenectomy aims to retain splenic immunologic function, while at the same time to decrease the rate of hemolysis. The long -term outcomes of patients with total or subtotal splenectomy for congenital hemolytic anemia, still remain unclear, but the majority of the studies showed a qualitative resolution of anemia and reduction of transfusion rate. Despite the well known advantages of conservative surgery, the optimal choice of treatment and outcomes should be confirmed with the patient.

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Section: The Role Of Splenectomy In Hereditary Spherocytosismentioning

confidence: 99%

Section: The Role Of Splenectomy In Hereditary Spherocytosismentioning

confidence: 99%

Section: The Role Of Splenectomy In Hereditary Spherocytosismentioning

confidence: 99%

See 1 more Smart Citation

Hereditary Spherocytosis - Diagnosis, Surgical Treatment and Outcomes. A Literature Review

Manciu¹,

Matei²,

Trandafir³

2017

chr

View full text Add to dashboard Cite

show abstract

“…Con la finalidad de evitar el riesgo de sepsis, se ha planteado como opción la esplenectomía parcial, en la cual alrededor del 90% del bazo es resecado. 67 Así, se reduce de modo sustancial el grado de destrucción eritrocitaria, lo que permite alcanzar con más facilidad un estado de hemólisis compensada, mientras que se deja tejido esplénico residual para mantener un grado aceptable de capacidad fagocítica. El principal reparo a esta técnica es que el tejido esplénico tiene una gran capacidad de regeneración, por lo que, con el paso de los años, se puede volver a la situación previa a la esplenectomía.…”

Section: Terapia Transfusional De Soporteunclassified

Esferocitosis hereditaria. Revisión. ParteII. Manifestaciones clínicas, evolución, complicaciones y tratamiento

2015

Arch Argent Pediat

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“…Efforts should be made to repair or preserve injured spleens and perform partial splenectomy in selected cases of congenital hemolytic anemias. 97 …”

mentioning

confidence: 99%

Splenosis and sepsis: The born-again spleen provides poor protection

Connell

Brunner

Kerr³

et al. 2011

Virulence

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Clinical and Hematologic Benefits of Partial Splenectomy for Congenital Hemolytic Anemias in Children

Abstract: In children with hereditary spherocytosis, a partial splenectomy appears to control hemolysis while retaining splenic function. In children with other congenital hemolytic anemias, a partial splenectomy appears to control symptoms of hypersplenism and splenic sequestration.

Cited by 88 publications

References 25 publications

Hereditary Spherocytosis - Diagnosis, Surgical Treatment and Outcomes. A Literature Review

Hereditary Spherocytosis - Diagnosis, Surgical Treatment and Outcomes. A Literature Review

Esferocitosis hereditaria. Revisión. ParteII. Manifestaciones clínicas, evolución, complicaciones y tratamiento

Splenosis and sepsis: The born-again spleen provides poor protection

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