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Blood, 15 January 2001, Vol. 97, No. 2, pp. 399-403
CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS
Long-term evaluation of the beneficial effect of
subtotal splenectomy for management of hereditary spherocytosis
Brigitte Bader-Meunier,
Frédéric Gauthier,
Frédérique Archambaud,
Thérèse Cynober,
Francoise Miélot,
Jean-Paul Dommergues,
Josiane Warszawski,
Narla Mohandas, and
Gil Tchernia
From the Département de Pédiatrie,
Laboratoire d'Hématologie, Service de médecine
nucléaire, Service d'épidémiologie et de
statistique, INSERM U 292, Hôpital Bicétre, Le Kremlin
Bicétre, France; Assistance Publique des Hôpitaux de Paris,
Faculté de Médécine Paris XI, Paris, France; and
Lawrence Berkeley National Laboratory, University of California,
Berkeley, CA.
Clinical manifestations of hereditary spherocytosis (HS) can be
abrogated by splenectomy. However, concerns exist regarding exposure of
patients to a lifelong risk for overwhelming infections and, to a
lesser extent, to vascular complications after total splenectomy. In
the search for alternative treatment modalities, we assessed, in a
previous pilot study, the potential usefulness of subtotal splenectomy
in a small population of patients. During a mean follow-up period of
3.5 years, subtotal splenectomy was shown to be effective in decreasing
the hemolytic rate, while maintaining the phagocytic function of the
spleen. In the current study, we evaluated the clinical and biologic
features of 40 patients with HS who underwent subtotal splenectomy and
were monitored for periods ranging from 1 to 14 years. The beneficial
effect of subtotal splenectomy included a sustained decrease in
hemolytic rate and a continued maintenance of phagocytic function of
the splenic remnant. However, mild-to-moderate hemolysis was persistent and accounted for secondary gallstone formation and aplastic crisis in
a small subset of patients. Surprisingly, regrowth of the remnant spleen did not seem to have a major impact on the beneficial outcomes of these individuals. Our results suggest that subtotal splenectomy appears to be a reasonable treatment option for management of patients
with HS, especially young children.
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