Clinical features
Clinical features include:
- characteristically occurs in middle age - median age at presentation is 55 years
- males affected five times more frequently than females
- symptoms are variable:
- gradual onset of fatigue
- recurrent infection, including mycobacterial infections
- left hypochondrial discomfort related to a massively enlarged spleen
- hepatomegaly is present in 50% of cases
- lymphadenopathy is uncommon
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