CD4+/CD8- T cell Large Granular Lymphocytic Leukemia: A rare Entity

Abstract

Large granular lymphocytic leukemia (LGL) is a well-recognized disorder of mature T-cells or NK cells. T-cell LGL leukemia (T-LGL) is characteristically a disorder of mature CD3+/CD8+ cytotoxic T-cells. Rare variants include CD 3+/CD4+/CD8-cases. To the best of our knowledge, 11 such cases (4 cases by Lima et al.[1] in 2003, 4 cases by Olteanu et al. in 2010,[2] 2 cases by Mutreja et al.[3] in 2014, and 1 case by Rabade et al.[4] in 2014) of T-LGL showing CD3+/CD4+/CD8-immunophenotype has been published in literature so far. There is a paucity of literature explaining the monoclonal expansion of CD3+/CD4+ T-LGL.[1] Unlike CD8+ T-LGL, CD4+ T-LGL does not show cytopenia, autoimmune phenotypes,[1],[5] or splenomegaly. However, CD4+ T-LGL is frequently associated with nonhematological malignancies.[1] Here, we report a case presenting with CD3+/CD4+/CD8-immunophenotype. Such immunophenotypic variant form of T-LGL cases should have a close clinical follow-up as they are prone to develop either simultaneously or months and years after, secondary hematological or nonhematological malignancies.[1]