[T-large granular lymphocyte leukaemia. An important differential diagnosis to Felty's syndrome].
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.
Moosig
F
F
Medizinische Klinik des Universitätsklinikum Schleswig-Holstein, Campus Kiel. f.moosig@med2.uni-kiel.de
Schoch
R
R
Kneba
M
M
D018501:Antirheumatic Agents / Q000627:therapeutic use; D000998:Antiviral Agents / Q000627:therapeutic use; D001172:Arthritis, Rheumatoid / Q000175:diagnosis / Q000188:drug therapy; D003937:Diagnosis, Differential; D005258:Felty Syndrome / Q000175:diagnosis; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007945:Leukemia, Lymphoid / Q000175:diagnosis* / Q000188:drug therapy; D015458:Leukemia, T-Cell / Q000175:diagnosis* / Q000188:drug therapy; D008727:Methotrexate / Q000627:therapeutic use; D014740:Vidarabine / Q000031:analogs & derivatives / Q000627:therapeutic use
D018501:Antirheumatic Agents; D000998:Antiviral Agents; D007166:Immunosuppressive Agents; D014740:Vidarabine; C024352:fludarabine; D008727:Methotrexate