T-Cell Large Granular Lymphocyte Leukemia: The Experience of a Single Center

• Autor(a) principal: Eira,Isabel M.
• Data de Publicação: 2018
• Outros Autores: Correia,Sandra I., Ângela,Cristina, Marques,Herlander
• Tipo de documento: Artigo
• Idioma: eng
• Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
• Texto Completo: http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-671X2018000100007
• topic: Autoimmune Diseases; Leukemia; Large Granular Lymphocytic; Lymphoma; T-Cell

Background: T-cell large granular lymphocyte (LGL) leukemia is a rare lymphoproliferative disease. It frequently involves the expansion of CD8+ cells, which may lead to cytopenias and often correlates with autoimmune disorders. Another form is the CD4+ LGL leukemia, which behaves more indolently but may associate with other neoplasia. Material and Methods: Retrospective and descriptive analysis of the 14 patients diagnosed with T-cell LGL leukemia in our center between 2002 and 2016, regarding gender/age; clinical presentation; immunophenotype; frequency of cytopenias; coexistent malignancies; autoimmune disorders and temporal relationship between both diagnosis; immunosuppressant treatment and its outcome. Results: Three patients had CD4+ LGL leukemia and eleven had CD8+ LGL leukemia. In the latter, neutropenia was the prevailing cytopenia (63.6%), followed by anemia (45.5%) and thrombocytopenia (36.4%). The most common symptoms were fatigue and recurrent bacterial infections; 35.7% presented with asymptomatic cytopenias. From patients with CD4+ LGL leukemia, one had colorectal cancer. Prevalence of autoimmune diseases was 35.7%; its diagnosis preceded the identification of LGL leukemia in all patients. Five patients required treatment; methotrexate was the most selected firstline immunosuppressant. Complete hematological response was achieved in two cases. Mortality rate was 14.3% at a median follow-up time of 2 years. Discussion and Conclusion: Clinical presentation and frequency of cytopenias were close to described in previous studies. The recurrent association with autoimmunity suggests the existence of common etiopathogenic features; chronic autoantigen stimulation might play a role in the onset of the clonal disease. Further studies are needed for determining the gold-standard approach of LGL leukemia.