Morgan J E, McCaul D S, Rodriguez F H, Abernathy D A, deShazo R D, Banks D E
Department of Medicine, Tulane University School of Medicine, New Orleans.
Chest. 1987 Oct;92(4):704-8. doi: 10.1378/chest.92.4.704.
A 74-year-old man presented with interstitial pulmonary disease which was proven to be alveolar septal amyloidosis by transbronchial biopsy. Multiple myeloma was diagnosed on the basis of monoclonal IgG-lambda protein in serum, monoclonal lambda light chains in urine, a bone marrow plasmacytosis of 22 percent, and serum IgA and IgM levels less than 100 mg/dl and 50 mg/dl, respectively. Appropriate investigations failed to show additional sites of deposition of amyloid. Analysis of fluid from bronchoalveolar lavage showed an increase in total cells recovered, a lymphocytosis with a ratio of T helper over T suppressor cells greater than that in peripheral blood, the presence of an IgG-lambda paraprotein, and an IgG/albumin ratio greater than that in serum. While plasma cells could not be identified in the recovered cell population, cultured cells from bronchoalveolar lavage fluid showed increased production of IgG. These findings provide evidence of an ongoing pulmonary immune response resulting in excess IgG-lambda protein in the pulmonary compartment, a factor which may contribute to the development of amyloidosis.
一名74岁男性因间质性肺疾病就诊，经支气管活检证实为肺泡间隔淀粉样变性。根据血清中单克隆IgG-λ蛋白、尿中单克隆λ轻链、骨髓浆细胞增多22%以及血清IgA和IgM水平分别低于100mg/dl和50mg/dl，诊断为多发性骨髓瘤。适当的检查未发现淀粉样蛋白沉积的其他部位。支气管肺泡灌洗液体分析显示回收的总细胞数增加，淋巴细胞增多，辅助性T细胞与抑制性T细胞的比例高于外周血，存在IgG-λ副蛋白，且IgG/白蛋白比例高于血清。虽然在回收的细胞群体中未识别出浆细胞，但支气管肺泡灌洗液体培养的细胞显示IgG产生增加。这些发现提供了正在进行的肺部免疫反应导致肺腔中IgG-λ蛋白过量的证据，这一因素可能有助于淀粉样变性的发展。
