Aggressive T‐cell large granular lymphocyte leukemia: A case report and review of the literature

Abstract

The majority of patients with T-cell large granular lymphocyte (LGL) leukemia will have an indolent clinical course. Herein, we report a case of an aggressive T-cell LGL leukemia in a previously healthy 42-year-old Caucasian male who presented with acute onset of Bsymptoms, hepatosplenomegaly, lymphocytosis, moderate anemia, and thrombocytopenia. Immunophenotypically, the malignant cells co-expressed CD3 + CD8 + CD56 + markers and the T-cell receptor beta (TCR b) gene demonstrated clonal rearrangement. The patient was treated with an intensive chemotherapeutic regimen (hyper-CVAD) and he achieved a complete remission. A systematic review of all available English literature revealed 12 welldescribed cases of aggressive T-cell LGL leukemia suggesting that this variant is a new and distinct entity in the spectrum of LGL disorders. Am. J. Hematol. 82:481-485, 2007.

CD56 Negative Aggressive T Cell Large Granular Lymphocytic Leukemia

“…LGL infiltration of lung as seen in our patient is uncommon and is reported in four cases of aggressive T-LGL in literature [7][8][9][10]. Immunophenotyping is essential for the diagnosis of LGL leukemias.…”

Aggressive LGL leukaemia presentation in old age

“…Four cases reported were characterised by pulmonary involvement, expression of CD95 (FAS) and high levels of circulating FAS ligand and were responsive to treatment [2], while the other cases including ours were not [3] [4] [5]. Apart from the hyper-leukocytosis and advanced age at presentation, the most striking aspect of our case was the simultaneous expression of stem cells markers (CD34+ , CD117+), myeloid marker (CD33+) in a cell with full T differentiation (CD3+, TCR Vβ3), NK markers (CD56+, CD16+) and late KIR expression (CD94 NK G2C).…”

Large granular lymphocytic leukaemia pathogenesis and management

“…Rarely, T-LGL leukaemia presents with a much more aggressive clinical behaviour, usually in younger individuals (Alekshun et al, 2007). Characteristically, patients have B symptoms, hepatosplenomegaly, cytopenias and LG lymphocytosis.…”

“…Most LGL leukaemias (80-90%) are CD3 positive with co-expression of CD8, CD16 and CD57, with CD56 and CD28 being negative (Semenzato et al, 1997 an underlying non-haemopoietic malignancy (Olteanu et al, 2010). CD56 expression is thought to define a subgroup of CD3 + T-cell LGL leukaemia with younger age of onset, more aggressive disease evolution and shorter survival (Gentile et al, 1994;Macon et al, 1996;Tordjman et al, 1996;Passetto Falcao et al, 2000;Alekshun et al, 2007). The rarer NK-LGL have a CD3 ) , CD56 + and/or CD16 + phenotype.…”

“…Data available from limited studies suggest superior approaches using more acute lymphoblastic leukaemia (ALL) -type induction therapy consolidated with stem cell transplantation in first remission (Alekshun et al, 2007). Stem cell transplantation has been described in a few anecdotal reports (Toze et al, 2000).…”