‘Non‐Tropical Idiopathic Splenomegaly’ (‘Primary Hypersplenism’): A Review of Ten Cases and their Relationship to Malignant Lymphomas
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Summary. The clinical, haematological and serological findings arc described of 10 patients believed to be suffering from a syndrome tentatively labelled ‘non‐tropical idiopathic splenomegaly’. The blood picture was characterized by leucopenia and neutropenia of severe degree, moderate thrombocytopenia and slight to moderate reticulocytosis. Abnormal or early forms of leucocytes were absent from peripheral blood films. All the patients were anaemic, although in only two was the total red‐cell volume subnormal. Five developed auto‐antibodies against red cells; one had hypogammaglobulinaemia. The spleen was grossly enlarged in all 10 patients and the liver was palpable in seven but there was no evidence of cirrhosis or portal hypertension.Splenectomy was carried out in nine of the patients, with sustained and striking clinical and haematological improvement in most instances. Four patients, however, remained neutropenic. The histological findings in the spleens were variable and in some of the patients the lymphoid foci were enlarged. There was, however, no evidence of overt malignant lymphoma.Four of the patients have died: two from illnesses unconnected with their splenomegaly; one from lymphosarcoma 8 months after splenectomy and one from auto‐immune haemolytic anaemia 41/2 years after splenectomy—this patient also showed evidence of lymphosarcoma at necropsy.The nature of the splenic enlargement remains obscure. While it is difficult absolutely to exclude an unusual and exaggerated response to an exogenous infection or infections, there was no evidence for this. An alternative possibility is that some at least of the patients were suffering from a type of auto‐immune disease, the main feature being exaggerated lympho‐reticular cellular proliferation, particularly within the spleen, rather than antibody formation. In some patients the lymphoproliferation was so marked as to suggest the development of a chronic ‘pre‐malignant’ lymphoma, predominantly (but not exclusively) affecting the spleen.
