Schönland S O, Bochtler T, Kristen A V, Ho A D, Hegenbart U
Medizinische Klinik V, Abteilung Hämatologie, Onkologie, Rheumatologie, Universitätsklinikum Heidelberg, Im Neuenheimer Feld 410, 69120, Heidelberg, Deutschland.
Pathologe. 2009 May;30(3):205-11. doi: 10.1007/s00292-009-1132-5.
Amyloidoses are protein-folding disorders in which soluble proteins are deposited as insoluble fibrillar aggregates due to a change in protein conformation. This might occur intra- or extracellularly, systemically or in a localized manner. The light chain type is the most common form of systemic amyloidoses and has the worst prognosis. The underlying disease is a monoclonal, mostly non-malignant plasma cell disorder. The causative treatment is the reduction of the amyloidogenic light chains with conventional or high-dose chemotherapy. Meanwhile, the"new drugs" used in multiple myeloma are also successfully applied. Early diagnosis is important to be able to treat patients effectively and to avoid further deterioration of organ function. Patients with newly diagnosed amyloidosis should be referred to a specialized center for consultation, diagnosis and treatment recommendation.
淀粉样变性是蛋白质折叠紊乱疾病，其中可溶性蛋白质由于蛋白质构象改变而沉积为不溶性纤维状聚集体。这可能发生在细胞内或细胞外、全身性或局部性。轻链型是全身性淀粉样变性最常见的形式，预后最差。潜在疾病是一种单克隆、大多为非恶性的浆细胞疾病。病因治疗是通过传统或大剂量化疗减少淀粉样生成轻链。同时，用于多发性骨髓瘤的“新药”也得到了成功应用。早期诊断对于有效治疗患者和避免器官功能进一步恶化很重要。新诊断的淀粉样变性患者应转诊至专业中心进行咨询、诊断和治疗建议。
