Cachat François, Meagher-Villemure Kathleen, Guignard Jean-Pierre
Department of Pediatrics, University Hospital, Lausanne, Switzerland.
Pediatr Nephrol. 2003 Aug;18(8):838-42. doi: 10.1007/s00467-003-1179-0. Epub 2003 Jun 12.
Lymphomatoid granulomatosis is a rare angiocentric and angiodestructive pulmonary angiitis considered as a variant of the lymphoproliferative disorder group. Patients with organ transplantation are at an increased risk for post-transplant lymphoproliferative disorders secondary to their immunosuppression. However, lymphomatoid granulomatosis has rarely been described in patients with renal transplantation. It often presents with severe pulmonary signs. We describe a case whose initial presentation was an isolated VIth nerve palsy. We review the radiological and pathological findings and discuss the etiopathogenesis and therapeutic options of this particular lymphoproliferative disorder. With careful and stepwise reduction in her immunosuppression, our patient showed a complete disappearance of her lymphomatoid granulomatosis, and she is clinically well more than 3 years after the diagnosis, with good kidney function.
淋巴瘤样肉芽肿病是一种罕见的以血管为中心和具有血管破坏性的肺血管炎,被认为是淋巴增殖性疾病组的一种变体。器官移植患者由于免疫抑制,发生移植后淋巴增殖性疾病的风险增加。然而,肾移植患者中很少描述有淋巴瘤样肉芽肿病。它常表现为严重的肺部症状。我们描述了一例最初表现为孤立性第六脑神经麻痹的病例。我们回顾了影像学和病理学发现,并讨论了这种特殊淋巴增殖性疾病的病因发病机制和治疗选择。随着对患者免疫抑制的谨慎逐步降低,我们的患者淋巴瘤样肉芽肿病完全消失,诊断后3年多来临床状况良好,肾功能正常。