EFFECT OF SPLENECTOMY ON PORPHYRIA ERYTHROPOIETICA

Abstract

Studies performed on a case of porphyria erythropoietica revealed a compensated hemolytic process. Cell survival studies, performed by the Ashby technique, showed the presence of erythrocytes of short life span, demonstrating an intracorpuscular defect in this patient. Fluorescence was found in a large percentage of the normoblasts of bone marrow aspirations, giving evidence that the bone marrow is the source of the excess porphyrins in this case. Splenectomy was performed in an attempt to reduce hemolysis, diminish erythropoiesis and porphyrin production, and thus secure palliation of the disease. The spleen and a liver biopsy were analyzed for porphyrins, and a considerable concentration of uroporphyrin and coproporphyrin was found in each organ. Following splenectomy there was a marked reduction in porphyrin excretion followed by a gradual rise approaching the preoperative levels. Some amelioration of the hydroa estivale was found in the first summer after splenectomy. The rising level of porphyrin excretion, however, suggests that the improvement will probably not be sustained. It is the opinion of the authors, however, that splenectomy should be considered in cases of porphyria erythropoietica in which increased hemolysis can be demonstrated, even if no anemia is present, because of possible variation in the response to the operation of individual patients.