Fournial F, Ravoteur B, Carre J C, Carriere J C, Delsol G, Bollinelli R
Poumon Coeur. 1979;35(2):97-100.
The authors reported two recent cases of broncho-pulmonary amylosis with monoclonal gammapathy. The first observation concerned a diffuse amylosis characterized by a bilateral pulmonary infiltration with pleural effusions and secretion of IgGlambda with a Bence Jones proteinuria. Pleural and pulmonary biopsies will provide the proof of amylosis. Evolution will rapidly be fatal in case of renal insufficiency. Post-mortem sampling will confirm the amyloid involvement (muscle, liver, kidney). The second observation described a localized amylosis developed in contact with a mediastino-pulmonary lymphoplasmocytic lymphoma with tumoral secretion of IgMlambda. Although pneumonectomy brought out the disappearance of the abnormal paraportein, death occurred within a few months, linked either to the spreading of the lymphomatous process or to the onset of a bacillary surinfection. The authors then recalled the main clinical and diagnostic data of the broncho-pulmonary amylosis. They underlined the importance of GLENNER'S work who, by proving the immunoglobulinic nature of amyloid, explained that in both cases, amylosis appeared as the consequences of an abnormal immunoglobulins synthesis.
作者报告了两例近期伴有单克隆丙种球蛋白病的支气管肺淀粉样变性病例。第一例观察病例为弥漫性淀粉样变性，其特征为双侧肺部浸润伴胸腔积液以及IgGλ分泌和本周氏蛋白尿。胸膜和肺部活检将提供淀粉样变性的证据。如果出现肾功能不全，病情进展将迅速致命。尸检取样将证实淀粉样物质的累及情况（肌肉、肝脏、肾脏）。第二例观察病例描述了一种局限性淀粉样变性，其与纵隔肺淋巴细胞性淋巴瘤相关，肿瘤分泌IgMλ。尽管肺切除术后异常副蛋白消失，但患者在数月内死亡，原因要么是淋巴瘤病情扩散，要么是发生了细菌性二重感染。作者随后回顾了支气管肺淀粉样变性的主要临床和诊断数据。他们强调了格伦纳工作的重要性，格伦纳通过证明淀粉样物质的免疫球蛋白性质，解释了在这两例病例中，淀粉样变性都是异常免疫球蛋白合成的结果。
