Red cell membrane disorders consist of HS where it is identified by clinical and laboratory diversity which is also revealed by recent molecular studies. A mutation is detected in one of the spherocytosis genes that causes defects in the erythrocyte membrane. The laparoscopic approach has been one of the new splenectomy surgical procedures for the treatment of HS. Partial splenectomy is performed in children to avoid post-splenectomy sepsis. Newer management helps understand the splenectomy protocol and suggests meticulous discussion between the patient, family, and healthcare provider. Hereditary spherocytosis (HS) or Minkowski-Chauffard syndrome is a familial genetic hemolytic condition that causes defects in the inner cytoskeleton of the erythrocyte membrane leading to anemia. The cells have a spherical rather than biconcave shape with no flexibility; it then becomes more susceptible to hemolysis as they cannot pass through the vessels without changing their shape. According to the opinion, the genetic defect is caused by the heterogeneous modification of one of the six genes that codes for the protein involved in the vertical associations that link the skeleton of the cell membrane to the lipid bilayer. Defects in the erythrocyte membrane skeleton are responsible for several hereditary hemolytic anemias associated with the abnormal shape of erythrocytes. Hemoglobin is released due to hemolysis. There are more reticulocytes present in the circulation and the bone marrow tries to produce more red blood cells than usual to prevent anemia. Hereditary spherocytosis (HS) occurs worldwide, but most commonly in Northern European descendants. It affects approximately 1 in 1,000-2,500 individuals, depending on the diagnostic principle in which... half of the paper... pleen and preserving enough tissue would help it perform the desired hematological and immune functions. Partial splenectomy decreases the symptoms of hypersplenism and splenic sequestration in children with hemoglobinopathies, while splenic function is less preserved than in children with spherocytosis. This can be supported by early studies where surgical procedure performed on 11 patients showed hemoglobin values increased while reticulocyte values decreased. This results in a direct increase in the number of erythrocytes after partial splenectomy. ] summarized their experience in Europe by explaining that irregular rates of splenic regrowth are not associated with recurrent hemolysis and the reasons behind this are unclear and may be due to parenchymal remodeling after partial resection.
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