A.Hereditary spherocytosis
B.Porphyria
C.Thalassemia
D.Sickle cell disease with large spleen
B.Porphyria
C.Thalassemia
D.Sickle cell disease with large spleen
Note:HEREDITARY
SPHEROCYTOSIS
The major clinical features of hereditary spherocytosis are anemia, splenomegaly, and
jaundice. Splenectomy reliably corrects the anemia, although the RBC defect and its consequent morphology persist. The operative risk is low. RBC survival after Splenectomy is normal or nearly
It should be noted that Cholecystectomy should not be performed without Splenectomy in any patient of hemolytic anemia, as intrahepatic gallstones may result.
Splenectomy in children should be postponed until age 4, if possible, to minimize the risk of severe infections with gram-positive encapsulated organisms. Polyvalent pneumococcal vaccine should be administered at least 2 weeks before splenectomy.
THALLESSIMIA
In-patient of thallessimia Splenectomy is required if the annual transfusion requirement, volume of RBCs per kilogram body weight per year increases by 50%.
SICKLE CELL ANEMIA
In sickle cell anemia repeated microinfarction in tissues occur due to sickling. Thus, the spleen is frequently infarcted within the first 18 to 36 months of life called autosplenectomy, causing susceptibility to infection, particularly from pneumococci. Acute venous obstruction of the spleen (splenic sequestration crisis leads to congestive splenomegaly), a rare occurrence in early childhood, may require emergency transfusion and/or splenectomy to prevent trapping of the entire arterial output in the obstructed spleen.
The major clinical features of hereditary spherocytosis are anemia, splenomegaly, and
jaundice. Splenectomy reliably corrects the anemia, although the RBC defect and its consequent morphology persist. The operative risk is low. RBC survival after Splenectomy is normal or nearly
It should be noted that Cholecystectomy should not be performed without Splenectomy in any patient of hemolytic anemia, as intrahepatic gallstones may result.
Splenectomy in children should be postponed until age 4, if possible, to minimize the risk of severe infections with gram-positive encapsulated organisms. Polyvalent pneumococcal vaccine should be administered at least 2 weeks before splenectomy.
THALLESSIMIA
In-patient of thallessimia Splenectomy is required if the annual transfusion requirement, volume of RBCs per kilogram body weight per year increases by 50%.
SICKLE CELL ANEMIA
In sickle cell anemia repeated microinfarction in tissues occur due to sickling. Thus, the spleen is frequently infarcted within the first 18 to 36 months of life called autosplenectomy, causing susceptibility to infection, particularly from pneumococci. Acute venous obstruction of the spleen (splenic sequestration crisis leads to congestive splenomegaly), a rare occurrence in early childhood, may require emergency transfusion and/or splenectomy to prevent trapping of the entire arterial output in the obstructed spleen.
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