PEDIATRICS Vol. 16 No. 3 September 1955, pp. 353-362
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SPLENECTOMY IN MEDITERRANEAN ANEMIA

David H. Clement M.D.1 and Max Taffel M.D.2

1 The Department of Pediatrics, the Yale School of Medicine, New Haven, Connecticut.
2 The Department of Surgery, the Yale School of Medicine, New Haven, Connecticut.

Although the primary defect in Mediterranean anemia is an inability to synthesize adequate amounts of hemoglobin, a hemolytic component eventually supervenes, associated with splenomegaly which in turn is augmented by the necessary transfusions.

When the disease bias been evident for some time, the spleen is greatly enlarged and the transfusion requirement is big, splenectomy may be effective in greatly reducing the frequency of transfusions to 25 to 50 per cent of the pre-splenectomy need.

Secondary benefits following splenectomy have included improvement in disposition, appetite and activity and lessened fatiguability. Overloading with iron is diminished, a feature theoretically diesirable in view of the ever-present hemochromatosis. Psychic trauma to the child and expenditure of the physician's time in transfusions are proportionately decreased.

Failure to benefit from splenectomy may be expected if the organ is removed early in the course of the disease before it has greatly enlarged and the transfusion need is great. It is also possible that the operation will accomplish little in the terminal phase of the disease when the liver is huge and the marrow over-extended.

Optimum time for splenectomy and the best criteria for the selection of patients remain to be evolved but we are convinced that removal of the spleen under appropriate circumstances is of substantial benefit to these patients. The procedure has been well tolerated in our small series. Complications have not been encountered, though thrombosis remains a threat in view of the high platelet counts encountered postoperatively. Early ambulation and even anticoagulant therapy (not yet employed by us) may be useful. Platelet counts should be followed closely.

Submitted on March 7, 1955
Accepted on April 23, 1955


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