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In no other form of splenomegaly are the indications for splenectomy so definite as in familial hemolytic jaundice. The disease is characterized by increased fragility of the red cells in hypotonic salt solution, reticulocytosis and splenic enlargement, with the presence of jaundice and an indirect positive Van den Bergh test. The underlying defect in red cell metabolism is congenital and hereditary, being transmitted according to mendelian principles.
The symptoms are characteristic, recurring in cycles and leading to so-called crises in which there are nausea, vomiting, increased jaundice and a progressive anemia. These symptoms, together with the family history and the laboratory observations, make the diagnosis almost obvious.
Splenectomy in these cases is clearly indicated and yields uniformly brilliant results. This is confirmed by our knowledge of the role of the spleen in the destruction of red blood cells. This destruction of red blood cells is a function of the reticuloendothelial
Ransohoff JL, O'Rourke W. TYPHOID ABSCESS OF THE SPLEEN IN HEMOLYTIC JAUNDICE. JAMA. 1940;114(26):2543-2544. doi:10.1001/jama.1940.62810260002007a