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Clinical features

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

Authoring team

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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