title: Long-term remission of T-cell large granular lymphocyte leukemia associated with rheumatoid arthritis after rituximab therapy.
abstract: T-cell large granular lymphocyte leukemia (LGLL) is a rare clonal disease often associated with rheumatoid arthritis (RA) and manifests chiefly as neutropenia and recurrent infections. Immunosuppressive agents are the mainstay of treatment, but long-term remissions are rare. We report 2 cases of LGLL in patients with RA successfully treated with rituximab, a monoclonal antibody specific of B cells and approved for treating RA. The first patient experienced a complete LGLL remission that was sustained during the 8-year follow-up after the first rituximab infusion. In the second patient, rituximab therapy was followed by immediate neutropenia recovery and then by marked shrinkage of the LGLL clone 1 year later. The paradoxical efficacy of this specific anti-B-cell drug on a monoclonal T-cell disease suggests that some cases of LGLL may be reactive manifestations of chronic autoantigen stimulation rather than true malignancies.
authors.affiliation: Service de Rhumatologie, Centre Hospitalier Régional et Universitaire de Brest, Brest, France. divi.cornec@chu-brest.fr
authors.forename: Divi
authors.initials: D
authors.lastname: Cornec
authors.forename: Valérie
authors.initials: V
authors.lastname: Devauchelle-Pensec
authors.forename: Sandrine
authors.initials: S
authors.lastname: Jousse-Joulin
authors.forename: Thierry
authors.initials: T
authors.lastname: Marhadour
authors.forename: Valérie
authors.initials: V
authors.lastname: Ugo
authors.forename: Christian
authors.initials: C
authors.lastname: Berthou
authors.forename: Nathalie
authors.initials: N
authors.lastname: Douet-Guilbert
authors.forename: Alain
authors.initials: A
authors.lastname: Saraux
mesh_terms: D000369:Aged, 80 and over; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D001172:Arthritis, Rheumatoid / Q000150:complications / Q000628:therapy*; D005260:Female; D006801:Humans; D007167:Immunotherapy; D054066:Leukemia, Large Granular Lymphocytic / Q000150:complications / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D012074:Remission Induction; D000069283:Rituximab; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000069283:Rituximab