Blood, 1960, Vol. 15, No. 2, pp. 197-211.
© 1960 American Society of Hematology, Inc.
The Role of Splenectomy in the Management
of Thalassemia
CARL H. SMITH 1,
MARION E. ERLANDSON 1,
GERTRUDE STERN 1, and
IRVING SCHULMAN 1
1 Department of Pediatrics, The New York Hospital-Cornell Medical Center.
A group of 58 children with severe Cooley’s anemia, half of them splenectomized, were reviewed from several of the more important clinical and
hematologic aspects.
Splenectomy eliminates the extracorpuscular mechanism responsible for
the accelerated destruction of normal donor red cells in the patient’s circulation. This represents a lasting improvement and accounts for an increased
longevity of normal blood supplements and a striking reduction of transfusion requirements. Less blood was required to maintain at least as good,
and at times improved, hemoglobin levels. Estimates of duration of survival
of the patient’s own erythrocytes suggest that the basic hemolytic defect is
not altered significantly by this operative procedure.
While transfusions and splenectomy promote the patient’s well being,
growth in height and sexual development are retarded. Secondary sexual
characteristics develop, if at all, later than in normal individuals and normal
menses are rare.
Cardiac failure occurred in 10 patients; all but one had been subjected to
splenectomy. This group was characterized by cardiac enlargement, multiple
arrhythmias and death with refractory heart failure, usually in the second
decade of life.
Cardiac complications are likely to be related to the heavy deposition of
iron in the myocardium, and are comparable in this respect to a similar
incidence in idiopathic hemochromatosis. One of the most serious problems
in the management of Cooley’s anemia is the prevention and control of
congestive heart failure. This will depend on the discovery of an effective
method by which the heart can be made to unload its iron deposits.
The increased susceptibility to infection following splenectomy in patients
with severe Cooley’s anemia indicates that the normal immunologic responses
inherent in the spleen remain intact despite the stress of a continuous
hemolytic process.
Submitted on April 27, 1959
Accepted on June 3, 1959
