Abstract

GARROTE SANTANA, Heidys et al. Long-term assessment of the partial splenectomy in patients with hereditary spherocytosis. Rev Cubana Hematol Inmunol Hemoter [online]. 2010, vol.26, n.1, pp.33-45. ISSN 0864-0289.

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.

Keywords : Hereditary spherocytosis; partial splenectomy; congenital hemolytic anemia.

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