Service de Rhumatologie, Centre Hospitalier Régional et Universitaire de Brest, Brest, France.
Blood. 2013 Aug 29;122(9):1583-6. doi: 10.1182/blood-2013-03-491464. Epub 2013 Jul 18.
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.
T 细胞大颗粒淋巴细胞白血病（LGLL）是一种罕见的克隆性疾病，常与类风湿关节炎（RA）相关，主要表现为中性粒细胞减少和反复感染。免疫抑制剂是主要的治疗方法，但长期缓解很少见。我们报告了 2 例 RA 患者的 LGLL 成功接受利妥昔单抗治疗的病例，利妥昔单抗是一种针对 B 细胞的单克隆抗体，已被批准用于治疗 RA。第一例患者在首次利妥昔单抗输注后的 8 年随访期间经历了完全的 LGLL 缓解。第二例患者接受利妥昔单抗治疗后立即出现中性粒细胞减少，随后 1 年后 LGLL 克隆明显缩小。这种针对 B 细胞的特异性药物对单克隆 T 细胞疾病的疗效矛盾表明，某些 LGLL 病例可能是慢性自身抗原刺激的反应性表现，而不是真正的恶性肿瘤。
