Mai Hoa L, Sheikh-Hamad David, Herrera Guillermo A, Gu Xin, Truong Luan D
Department of Pathology, Ben Taub General Hospital and Baylor College of Medicine, Houston, TX 77030, USA.
Am J Surg Pathol. 2003 Apr;27(4):541-5. doi: 10.1097/00000478-200304000-00016.
It is not well recognized that monoclonal immunoglobulin heavy chains or their fragments can be amyloidogenic. Amyloidosis due to heavy chains, referred to as AH amyloidosis, is rare with only three cases previously reported. An additional case of AH amyloidosis is reported. To the best of our knowledge, this is the first case of IgM heavy chain amyloidosis. A 59-year-old man presented with nephrotic syndrome. Immunoelectrophoresis detected a monoclonal IgM lambda in his serum and free lambda light chains in his urine. A renal biopsy showed amyloidosis, in which the amyloid deposits stained only for mu heavy chain by immunofluorescent and immunoelectron microscopic studies. This case suggests that monoclonal immunoglobulin heavy chains can be amyloidogenic. Furthermore, this type of amyloidosis shares the same morphologic features with other types of amyloidosis and can involve the kidney to produce nephrotic syndrome.
单克隆免疫球蛋白重链或其片段可具有淀粉样变性，这一点尚未得到广泛认可。由重链引起的淀粉样变性，称为AH淀粉样变性，非常罕见，此前仅报道过3例。本文报道了另外1例AH淀粉样变性病例。据我们所知，这是首例IgM重链淀粉样变性病例。一名59岁男性表现为肾病综合征。免疫电泳在其血清中检测到单克隆IgM λ，尿液中检测到游离λ轻链。肾活检显示淀粉样变性，免疫荧光和免疫电子显微镜研究表明，淀粉样沉积物仅对μ重链染色。该病例表明单克隆免疫球蛋白重链可具有淀粉样变性。此外，这种类型的淀粉样变性与其他类型的淀粉样变性具有相同的形态学特征，可累及肾脏导致肾病综合征。
