Lemal Richard, Chaleteix Carine, Minard Patrice, Roche Corinne, Bay Jacques-Olivier, Tournilhac Olivier, Lamy Thierry
Service de Thérapie Cellulaire et d'Hématologie Clinique Adulte, CHU Clermont-Ferrand Hôpital Estaing, Université d'Auvergne CREaT - EA 7283, INSERM CIC-501, Clermont-Ferrand, F-63000, France.
Service d'Oncologie, CH Vichy, Vichy, France.
Leuk Res Rep. 2013 Apr 2;2(1):32-3. doi: 10.1016/j.lrr.2013.02.004. eCollection 2013.
Large granular lymphocytic (LGL) leukemia is an uncommon clonal lymphoproliferative disorder. Lambert-Eaton Myasthenic Syndrome (LEMS) is a rare neuromuscular autoimmune disease caused by pathogenic autoantibodies targeting the voltage-gated calcium channels (VGCC) on the presynaptic nerve terminal. We here describe the case of a 77-year old patient with LGL leukemia, associated with a seropositive and symptomatic LEMS and a seronegative rheumatoid arthritis. LGL leukemia treatment clearly improved LEMS symptoms, and led to anti-VGCC antibodies value decrease. To our knowledge, this is the first ever described association between LGL leukemia and LEMS.
大颗粒淋巴细胞（LGL）白血病是一种罕见的克隆性淋巴细胞增殖性疾病。兰伯特-伊顿肌无力综合征（LEMS）是一种罕见的神经肌肉自身免疫性疾病，由针对突触前神经末梢电压门控钙通道（VGCC）的致病性自身抗体引起。我们在此描述了一例77岁的LGL白血病患者，其伴有血清学阳性且有症状的LEMS以及血清学阴性的类风湿性关节炎。LGL白血病的治疗明显改善了LEMS症状，并导致抗VGCC抗体值下降。据我们所知，这是首次报道的LGL白血病与LEMS之间的关联。
