Galtier Jean, Duval Fanny, Machelart Irène, Greib Carine, Lazaro Estibaliz, Pellegrin Jean-Luc, Viallard Jean-François, de la Tour Regis Peffault, Rivière Etienne
Hematology Department Haut-Leveque Hospital University Hospital Center of Bordeaux Pessac France.
Neurology Department Pellegrin Hospital University Hospital Center of Bordeaux Bordeaux France.
EJHaem. 2021 Sep 29;2(4):834-837. doi: 10.1002/jha2.244. eCollection 2021 Nov.
Paroxysmal nocturnal hemoglobinuria (PNH) is a very rare clonal autoimmune disease manifesting with hemolysis, thrombosis, or bone marrow failure. We present an atypical association of myasthenia gravis, aplastic anemia, and PNH occurring years after thymectomy. While this association might be extremely rare, it may not be coincidental as there is a common pathophysiology between PNH and aplastic anemia, with the latter reported in several thymoma/thymectomy cases. Eculizumab was introduced with good efficacy and without safety concern in our patient, leading to long-term control of PNH without worsening of myasthenia gravis.
阵发性睡眠性血红蛋白尿症（PNH）是一种非常罕见的克隆性自身免疫性疾病，表现为溶血、血栓形成或骨髓衰竭。我们报告了一例重症肌无力、再生障碍性贫血和PNH的非典型关联，该关联在胸腺切除术后数年出现。虽然这种关联可能极为罕见，但可能并非巧合，因为PNH和再生障碍性贫血之间存在共同的病理生理学，后者在几例胸腺瘤/胸腺切除病例中已有报道。在我们的患者中，使用依库珠单抗疗效良好且无安全问题，可长期控制PNH，而不会使重症肌无力恶化。
