Patuzzo G, Tinazzi E, Micheletti M, Puccetti A, Lunardi C
Clinical Immunology and Allergy Unit Department of Medicine University of Verona 37134 Verona, Italy. E-mail:
Department of Medicine, University of Verona, Verona, Italy.
Eur Ann Allergy Clin Immunol. 2016 Mar;48(2):55-7.
Primary intestinal lymphangiectasia (PIL) is rare disorder characterized by congenital malformation or obstruction of intestinal lymphatic drainage; it is responsible for protein losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. A low-fat diet associated with medium-chain triglyceride supplementation is the cornerstone of PIL management. The administration of intravenous immunoglobulins does not always lead to satisfactory plasma levels and therefore the replacement therapy with immunoglobulins is controversial. We describe here the case of a patient with PIL and severe hypogammaglobulinemia treated with immunoglobulins. The striking aspect of this case is the clinical and serological benefit obtained with the subcutaneous compared to the intravenous immunoglobulins administration.
原发性肠淋巴管扩张症（PIL）是一种罕见的疾病，其特征为肠道淋巴引流的先天性畸形或梗阻；它会导致蛋白丢失性肠病，进而引起淋巴细胞减少、低白蛋白血症和低丙种球蛋白血症。低脂饮食联合中链甘油三酯补充是PIL治疗的基石。静脉注射免疫球蛋白并不总能使血浆水平达到满意效果，因此免疫球蛋白替代疗法存在争议。我们在此描述一例接受免疫球蛋白治疗的PIL合并严重低丙种球蛋白血症患者的病例。该病例的显著之处在于，与静脉注射免疫球蛋白相比，皮下注射免疫球蛋白在临床和血清学方面均取得了良好效果。
