Pick A I, Fröhlichmann R, Lavie G, Duczyminer M, Skvaril F
Acta Haematol. 1981;66(3):154-67. doi: 10.1159/000207123.
Amyloidosis associated with plasma cell dyscrasia (AAPCD) is a relatively rare clinical entity (4% of our patients with PCD) and its early recognition and distinction from multiple myeloma (MM) may be of great therapeutic and prognostic significance. Laboratory parameters, such as concentrations of normal polyclonal Ig, Bence-Jones proteins and serum monoclonal components (MC) showed in our patients lower MC concentrations than in MM, lambda-L-chains and of gamma-H-chains predominating. Sequential skeletal X-ray studies and bone marrow morphology remain essential diagnostic procedures. Due to the lack of efficient therapeutic agents for AAPCD and the great progress achieved in recent years in the treatment of secondary amyloidosis, the immunochemical analysis of the isolated amyloid fibril as well as of the surrounding 'ground substance' should be pursued in AAPCD. Our data support previous observations, that in AAPCD the amyloid fibril subunit is an L-chain fragment predominantly derived from lambda-L-chains which originates from the same clone as the MC. The localization of an enzymatic cleavage point on the L-chain, the detection of a specific proteolytic enzyme and the identification of additional components in the amyloid substance, may further elucidate the etiopathogenesis of AAPCD.
浆细胞发育异常相关性淀粉样变性（AAPCD）是一种相对罕见的临床病症（占我们浆细胞发育异常患者的4%），早期识别并将其与多发性骨髓瘤（MM）区分开来可能具有重大的治疗和预后意义。实验室参数，如正常多克隆免疫球蛋白、本周蛋白和血清单克隆成分（MC）的浓度，在我们的患者中显示出比MM更低的MC浓度，以λ轻链和γ重链为主。连续的骨骼X线检查和骨髓形态学仍是重要的诊断程序。由于缺乏针对AAPCD的有效治疗药物，且近年来继发性淀粉样变性的治疗取得了很大进展，因此在AAPCD中应进行分离的淀粉样原纤维以及周围“基质”的免疫化学分析。我们的数据支持先前的观察结果，即在AAPCD中，淀粉样原纤维亚基是主要源自λ轻链的轻链片段，该轻链与MC来自同一克隆。轻链上酶切位点的定位、特定蛋白酶的检测以及淀粉样物质中其他成分的鉴定，可能会进一步阐明AAPCD的病因发病机制。
