[T-large granular lymphocyte leukaemia. An important differential diagnosis to Felty's syndrome]

Z Rheumatol. 2006 Sep;65(5):447-51. doi: 10.1007/s00393-005-0013-4.
[Article in German]


T-Large Granular Lymphocyte (T-LGL) leukaemia is a rare clonal disease characterized by neutropenia and/or anaemia. Because of its strong association with rheumatoid arthritis (RA), T-LGL leukaemia is an important differential diagnosis to Felty's syndrome. This differentiation might be especially difficult since, in severe RA with extraarticular manifestations, there is often an expanded memory effector T-cell population which can hardly be separated from T-LGL leukaemia cells by means of immunophenotyping. The main criterion for T-LGL leukaemia is the detection of a clonal T-cell-receptor rearrangement by PCR. First-line therapy consists of weekly low-dose methotrexate. Alternatively, other immunosuppressives or cytotoxic agents can be useful. There are very limited data from therapy studies. The German CLL study group has initiated a protocol using parenteral low-dose methotrexate as first-line therapy and fludarabine as second-line medication.

Publication types

  • English Abstract
  • Review

MeSH terms

  • Antirheumatic Agents / therapeutic use
  • Antiviral Agents / therapeutic use
  • Arthritis, Rheumatoid / diagnosis
  • Arthritis, Rheumatoid / drug therapy
  • Diagnosis, Differential
  • Felty Syndrome / diagnosis
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Leukemia, Lymphoid / diagnosis*
  • Leukemia, Lymphoid / drug therapy
  • Leukemia, T-Cell / diagnosis*
  • Leukemia, T-Cell / drug therapy
  • Methotrexate / therapeutic use
  • Vidarabine / analogs & derivatives
  • Vidarabine / therapeutic use


  • Antirheumatic Agents
  • Antiviral Agents
  • Immunosuppressive Agents
  • Vidarabine
  • fludarabine
  • Methotrexate