Successful allogeneic peripheral blood stem cell transplantation for an aggressive variant of T-cell large...

The aggressive variant of large granular lymphocyte (LGL) leukemia is very rare and the prognosis of this disease is poor. A 47-year-old woman with progressive pancytopenia and severe liver damage visited our institute. Upon hospitalization, about 30% LGL was detected in her peripheral blood and bone marrow samples. Flow cytometry was conducted to analyze lymphocytes in the bone marrow, which revealed the presence of CD3 and T-cell receptor (TCR) α/β and absence of CD4, CD8, CD16, CD56, CD22, CD79a, and terminal deoxynucleotidyl transferase (TdT). Southern blotting was performed, which revealed the presence of rearrangement of TCR-Cβ1 and Jγ. We made a diagnosis of the aggressive variant of T-LGL leukemia, and performed myeloablative allogeneic peripheral stem cell transplantation (allo-HSCT) from an HLA-matched sibling for primary refractory disease of CHOP and hyper CVAD therapy. She is alive in remission with donor-derived T-LGL lymphocytosis in peripheral blood for 7 years after allo-HSCT. Overall, Allo-HSCT could be active against the aggressive variant of LGL leukemia and induce graft-versus-leukemia effect.