Martín Reyes G, García González I, Alférez M J, Martínez González J M, Frutos M A
Servicio de Nefrología, Hospital Regional Universitario Carlos Haya, Avda. Carlos Haya, 82, 29010 Málaga.
Nefrologia. 2001 Mar-Apr;21(2):213-6.
A 45 year old man with IgA-Kappa myeloma had adult Fanconi Syndrome. Examination of renal biopsy revealed lesions in proximal tubules without glomerular lesions and without intratubular casts. By electron microscopy cytoplasmic crystalline inclusions were observed in renal proximal tubular epithelium. Increased plasma cells (28%) in bone marrow aspiration also contained crystalline inclusions. The treatment of myeloma produced partial remission of proliferative disease and Fanconi syndrome. We discuss the pathogenesis of Fanconi syndrome induced by light chains as well as the composition of crystalline deposits and the effects of treatment on Fanconi Syndrome.
一名患有IgA-κ型骨髓瘤的45岁男性患有成人范科尼综合征。肾活检显示近端肾小管有病变，无肾小球病变且无肾小管内管型。通过电子显微镜观察，在肾近端肾小管上皮细胞中发现了细胞质晶体包涵体。骨髓穿刺中浆细胞增多（28%），其中也含有晶体包涵体。骨髓瘤的治疗使增殖性疾病和范科尼综合征部分缓解。我们讨论了轻链诱导的范科尼综合征的发病机制、晶体沉积物的组成以及治疗对范科尼综合征的影响。
