Clinical features | One-third asymptomatic Two-thirds symptomatic Cytopenias, splenomegaly, rheumatoid arthritis (~25% of patients). | Symptomatic, usually with B symptoms (fever, sweats, weight loss) Lymphadenopathy, hepatosplenomegaly. | Symptomatic, usually with B symptoms (fever, sweats, weight loss) Lymphadenopathy, hepatosplenomegaly | Most patients are asymptomatic; about 40% with signs (cytopenias, vasculitis, neuropathy, splenomegaly) |
Treatment approach | Observation or immunosuppressive therapy, if required | Acute lymphoblastic leukemia-type therapy | Acute lymphoblastic leukemia-type therapy | Observation or immunosuppressive therapy if required |