clinical features

Last reviewed 01/2018

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