2016PubMedOpen access

Subtotal Splenectomy in Hereditary Spherocytosis - Advantages and Disadvantages.

Cătălina Bică, L Cismaru, L Stănescu, Marius Bică, D. Marinescu

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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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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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Available 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.

Key concepts: Hereditary spherocytosis, Splenectomy, Medicine, Spherocytosis, Hemolysis, Blood Disorder, Anemia, Hemolytic anemia

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