Syndrome of Hypogammaglobulinemia, Splenomegaly and Hypersplenism

Two cases of acquired idiopathic hypogammaglobulinemia associated with splenomegaly and hemolytic anemia due to hypersplenism have been reported. Splenomegaly and hypersplenism appears to be the result of reticulum cell hyperplasia caused by the lack of gamma globulin and resultant repeated infections. Both the cases had splenectomy with marked hematologic improvement. A review of the literature indicates that this syndrome is relatively common, in comparison to the incidence of total number of acquired hypogammaglobulinemia that has been reported in the literature so far. It also seems very important to recognize it because of the obvious therapeutic implications. Serum gamma globulin level should be determined in cases with unexplained hepatosplenomegaly, hypersplenism and hemolytic anemia.