Non-tropical 'idiopathic splenomegaly': a follow-up study of ten patients described in 1969

The later history is described of four of 10 patients who were reported in 1969 as suffering from non-tropical 'idiopathic splenomegaly'. Two of the four patients developed malignant lymphomas 6 years and 2 years, respectively, after splenectomy but the two other patients have lived for 17 and 15 years, respectively, without developing any signs of a malignant tumour. Thus, four of the original 10 patients have developed malignant's lymphomas. The histology of the patients' spleens has been reviewed in the light of their clinical history, but no criteria have been found which are of clear prognostic value. However, with hindsight it was possible to recognize some cytological abnormality in the spleens in three out of the four patients who developed malignant lymphomas, and it appears that unless the cell population in both the red and white pulp is strictly normal, the patient is likely to develop a malignant lymphoma. The tumours which subsequently developed showed no special or characteristic features. The title 'idiopathic splenomegaly' is clearly unsatisfactory but the alternative 'chronic lymphocytic lymphoma with hypersplenism', although appropriate for the illness of the patients who developed malignant lymphomas, is inappropriate for those in which the pathological process seems not to be neoplastic.