[Effectiveness of partial splenectomy in the treatment of hereditary spherocytosis].

OBJECTIVES

To evaluate the hematological and clinical response to partial splenectomy in a group of children with hereditary spherocytosis.

MATERIAL AND

METHODS

We analyzed the main hematological and clinical features in 13 patients with hereditary spherocytosis submitted to partial splenectomy. The diagnosis of the disease and the hematological studies was made according to standard methods. To establish the inheritance pattern we also studied both parents. During the surgical procedure the upper two thirds of the spleen were removed and the lower pole was preserved. We use prophylactic penicillin (250 mg twice a day) during 3 years.

RESULTS

The diagnosis of the disease was made during the first year of life in 11 patients, 76.9% had neonatal hyperbilirrubinemia, 53.8% showed hepatomegaly and in 69.2% we observed splenomegaly. 84.6% received blood transfusion. The hemoglobin level was significantly increased (p = 0.04) and the reticulocytes significantly diminished (p = 0.01) after splenectomy. No hemolytic crises, blood cell transfusion requirement neither infection was observed after surgery.

CONCLUSIONS

Although the number of patients and the post-surgical follow-up is reduced, the hematological recovery observed in our cases suggest that partial splenectomy is a beneficial surgical procedure for the treatment of the typical and severe form of hereditary spherocytosis.