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

Abstract

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.