title: Atypical aleukemic presentation of large granular lymphocytic leukemia: a case report.
abstract: Large granular lymphocytic leukemia (LGLL) is a rare lymphoproliferative disorder of transformed natural killer or T-cells attributed to chronic exposure to the proinflammatory cytokine IL-15. Diagnosis of the majority of T-cell LGLL is established by documenting clonal large granular lymphocytes (LGLs) in peripheral blood, by morphology and immunophenotype. The proteasome inhibitor bortezomib is known to target molecular pathways downstream of the IL-15 receptor signaling and has been proposed as a therapy in these patients. We report an uncommon presentation of LGLL with chronic neutropenia lacking typical blood LGLs, which failed to respond to bortezomib but obtained a very good partial remission with a classical methotrexate regimen.
authors.affiliation: Department of Hematology, Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania; Laboratory of Immunology and Immunotherapy, University Hospital Brest.
authors.forename: Cristina
authors.initials: C
authors.lastname: Bagacean
authors.affiliation: Department of Hematology, Institute of Cancerology and Hematology, Brest University Medical School, Brest, France.
authors.forename: Adrian
authors.initials: A
authors.lastname: Tempescul
authors.affiliation: Department of Hematology, Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania; Department of Hematology, Ion Chiricuta Oncology Institute, Cluj-Napoca.
authors.forename: Mariana
authors.initials: M
authors.lastname: Patiu
authors.affiliation: Department of Hematology, Ion Chiricuta Oncology Institute, Cluj-Napoca.
authors.forename: Bogdan
authors.initials: B
authors.lastname: Fetica
authors.affiliation: Department of Hematology, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.
authors.forename: Horia
authors.initials: H
authors.lastname: Bumbea
authors.affiliation: Department of Hematology, Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania; Department of Hematology, Ion Chiricuta Oncology Institute, Cluj-Napoca.
authors.forename: Mihnea
authors.initials: M
authors.lastname: Zdrenghea
keywords: Felty; bortezomib; large granular lymphocytes; lymphoproliferative; neutropenia