RESUMO
The association of T-cell large granular lymphocyte (LGL) leukemia and rheumatoid arthritis is well described and it is now recognized that these patients and patients with Felty's syndrome represent different aspects of a single disease process. Most patients have rheumatoid arthritis at the time of diagnosis of LGL leukemia. This is the first detailed report of the development of rheumatoid arthritis after the diagnosis and treatment of LGL leukemia as well as the first report of rheumatoid arthritis that occurred in association with deoxycoformycin treatment. It is likely that the beneficial sustained normalization of neutrophil counts as a result of deoxycoformycin treatment played a significant role in the development of this complication. Hematological improvement occurred despite molecular genetic evidence of persistence of the abnormal T-cell clone. The role of the clonally expanded T cells in the pathogenesis of neutropenia and rheumatoid arthritis is discussed.
Assuntos
Antibióticos Antineoplásicos/efeitos adversos , Síndrome de Felty/induzido quimicamente , Leucemia Linfoide/tratamento farmacológico , Pentostatina/efeitos adversos , Antibióticos Antineoplásicos/uso terapêutico , Ensaios Clínicos Fase II como Assunto , Células Clonais , Síndrome de Felty/patologia , Rearranjo Gênico do Linfócito T , Genes Codificadores dos Receptores de Linfócitos T/genética , Antígeno HLA-DR4/genética , Antígeno HLA-DR4/imunologia , Teste de Histocompatibilidade , Humanos , Masculino , Pessoa de Meia-Idade , Neutropenia/tratamento farmacológico , Neutropenia/imunologia , Neutropenia/patologia , Pentostatina/uso terapêutico , Reação em Cadeia da PolimeraseRESUMO
This prospective study documents the haematological responses in 300 rheumatoid patients (RA) treated with sulphasalazine (SASP) for between 1 and 9 years. It also examines the effect of SASP on the total white cell and platelet counts over 2 years in relation to disease activity in 80 RA patients. Neutropenia occurred in six (2%) (three severe--neutrophil count less than 0.8 X 10(9)/l) after 3 and 12 weeks. The drug was withdrawn in six immediately and in one patient after 21 months when the neutrophil count fell to 0.7 X 10(9)/l. An additional 11 (3.7%) developed mild or transient leucopenia between 2 weeks and 24 months, and eight continued therapy. Thrombocytopenia occurred in one patient at 18 weeks associated with other reactions. Four with Felty's syndrome developed a further fall in the total WBC associated with thrombocytopenia in two. A rise in mean cell volume was common (72%), and macrocytosis (MCV greater than 98 fl) occurred in 27 (9%). Macrocytic anaemia was rare (less than 1%). All haematological problems were reversible. In 80 patients treated with SASP for 2 years there was a significant fall in the median white cell and platelet counts at 3 months associated with improvement in disease activity.
