Hypersplenism is a clinical syndrome; it does not imply a specific causal mechanism. It has the following characteristic features:
1 enlargement of the spleen;
2 reduction in one or more of the cell lines in the peripheral blood;
3 normal or hyperplastic cellularity of the bone marrow, often with orderly maturation of earlier stages but paucity of more mature cells;
4 premature release of cells into peripheral blood, resulting in reticulocytosis and/or large immature platelets;
5 increased splenic red cell pool, decreased red cell survival and increased splenic pooling of platelets with shortening of their lifespan.
The diagnosis of hypersplenism is ultimately confirmed by the response to splenectomy, although an immediate remission may be followed in the longer term by partial relapse.
Most of the diseases listed in Table 21.1 can give rise to secondary hypersplenism. In these conditions, the haematological features of hypersplenism may be obscured or dominated by the primary disease, especially if it involves the marrow. Hyper-splenism also occurs as a primary event, due to an unknown pathogenetic stimulus. It is sometimes termed primary splenic hyperplasia, splenic neutropenia or splenic anaemia, and it includes those cases of non-tropical primary splenomegaly in which there is no firm evidence for an underlying lymphoma.
Table 21.1 Causes of splenomegaly.
Haematological Acute leukaemia Chronic myeloid leukaemia* Chronic lymphocytic leukaemia
Malignant lymphomas (some cases present as 'non-tropical primary splenomegaly')* Chronic (primary) myelofibrosis* Polycythaemia vera Thrombocythaemia (some cases) Hairy cell leukaemia*
Gaucher's disease*, Niemann-Pick disease, Langerhans cell histocytosis X* Primary splenic hyperplasia 'Non-tropical splenomegaly' Splenic anaemia/neutropenia Thalassaemia
Sickle-cell disease, HbSC disease and other haemoglobinopathies Haemolytic anaemias
Acute anaemia Megaloblastic anaemia
Acute infections: septicaemia, typhoid, infectious mononucleosis, cytomegalovirus
Subacute and chronic infections: tuberculosis, syphilis, brucellosis, subacute bacterial endocarditis, AIDS Tropical parasitic infections (tropical splenomegaly*): malaria*, leishmaniasis*, schistosomiasis*, trypanosomiasis Collagen diseases: systemic lupus erythematosus, rheumatoid arthritis (Felty) Sarcoidosis Amyloidosis Cysts
Haemangiomas Carcinoma (rare) Congestive splenomegaly Portal hypertension Cirrhosis
Splenic/portal/hepatic vein obstruction Congestive cardiac failure
*Common causes of splenomegaly.
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