Resumo

EIRA, Isabel M.; CORREIA, Sandra I.; ANGELA, Cristina  e  MARQUES, Herlander. T-Cell Large Granular Lymphocyte Leukemia: The Experience of a Single Center. Medicina Interna [online]. 2018, vol.25, n.1, pp.23-29. ISSN 0872-671X.

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.

Palavras-chave : Autoimmune Diseases; Leukemia; Large Granular Lymphocytic; Lymphoma; T-Cell.

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