Initial assessment of the beneficial effect of partial splenectomy in
hereditary spherocytosis
G Tchernia, F Gauthier, F Mielot, JP Dommergues, J Yvart, JA Chasis and N Mohandas
Laboratoire d'Hematologie, Hopital Bicetre, Kremlin Bicetre, France.
Clinical manifestations of hereditary spherocytosis (HS), the most common
red blood cell (RBC) membrane disorder, can be abrogated or markedly
reduced by splenectomy. However, concerns regarding risks from overwhelming
infections after splenectomy have restricted its use, especially in
children. This study was designed to determine if partial splenectomy can
decrease the hemolytic rate while maintaining phagocytic function of the
spleen. Partial splenectomy was performed in 11 children (age 2 to 13) with
HS. The effect on hemolytic rate was assessed by comparing the presurgical
and postsurgical values for hemoglobin, reticulocyte number, and RBC life
span. The residual splenic phagocytic function was assessed using
technetium 99m scans and by enumerating the percentage of pitted RBCs in
circulation. There were no complications from the surgical procedure in any
of the 11 individuals. Following partial splenectomy, hemoglobin increased
on the average by 3 g/dL, reticulocyte count decreased by 300 x 10(6)/L,
and RBC life span was substantially prolonged. Normal technetium uptake was
noted in the splenic remnant and the percentage of pitted RBCs was in the
normal range. Partial splenectomy is effective in decreasing the hemolytic
rate while maintaining residual splenic phagocytic function of the spleen
in HS. We conclude that the use of this procedure as treatment for RBC
membrane disorders warrants consideration, especially in infants under 5
years of age who need frequent transfusions.
Volume 81,
Issue 8,
pp. 2014-2020,
04/15/1993
Copyright © 1993 by The American Society of Hematology
