Subtotal Splenectomy in Hereditary Spherocytosis - Advantages and Disadvantages

ABSTRACT: Hereditary spherocytosis (HS) represents the most frequent hemolytic anemia in Central and Northern Europe consisting in an inherited abnormality of the red blood cell (RBC) membrane. It is usually transmitted as an autosomal dominant disorder; 25% of cases are without family history. Sple...

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Detalles Bibliográficos
Autores principales: BICĂ, C., CISMARU, L., STĂNESCU, L., BICĂ, M, MARINESCU, D.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medical University Publishing House Craiova 2016
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6269614/
https://www.ncbi.nlm.nih.gov/pubmed/30581596
http://dx.doi.org/10.12865/CHSJ.42.04.11
Descripción
Sumario:ABSTRACT: Hereditary spherocytosis (HS) represents the most frequent hemolytic anemia in Central and Northern Europe consisting in an inherited abnormality of the red blood cell (RBC) membrane. It is usually transmitted as an autosomal dominant disorder; 25% of cases are without family history. Splenectomy is the classical conception and it can cure hemolysis, being the treatment of choice for moderate to severe forms of HS. A new approach is accepted nowadays, subtotal splenectomy, thus eliminating the lifelong risk of postsplenectomy infections. We present two cases of HS treated by subtotal splenectomy, alongside the advantages and disadvantages of this therapy.

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