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Evaluación a largo plazo de la esplenectomía parcial en pacientes con esferocitosos hereditaria

Keywords: hereditary spherocytosis, partial splenectomy, congenital hemolytic anemia.

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Abstract:

the effectiveness of partial splenomegalia (ps) was assessed in 17 patients with hereditary spherocytosis seen in the hematology and immunology institute. age at diagnosis was of 6.71 ± 5.38 months. all of them had splenomegalia. a patient had vesicular lithiasis before intervention. criteria to presence of splenomegalia were: transfusion requirements (822.4 %), chronic anemia and splenomegalia (11.7 %) and splenomegalia (5.9 %). age at moment of ps was of 7.0 ± 2.6 years. hemoglobin (hb) was low in the 94.1 % of patients; reticulocytes increased in the 100 % and the total and indirect bilirubin was high in the 76.5 % and the 88.2 %, respectively. current mean age of patients is 16.24 ± 4.26 years with a course time of 9.24 ± 4.47 years. postoperative laboratory variables showed a significant increase of hb (p= 4 × 10-9) and a decrease of reticulocytes (p= 0.003). trend in time of hb remained stable in levels achieved after intervention in all patients with more than 10 years of operated on, but not for reticulocytes. two patients showed a growing of splenic fragment; one was associated with a poor clinical-hematological response. there not sepsis or thromboembolism complications after procedure.

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