Schwartz R S, Cohen J R, Schrier S L
Arch Intern Med. 1979 Oct;139(10):1144-7.
Two patients with progressive primary amyloidosis, monoclonal serum and urinary proteins, multiple organ involvement, and nephrotic syndrome were treated with melphalan and prednisone for one year. In one patient, splenomegaly and nephrotic syndrome rapidly responded to therapy but massive hepatomegaly responded slowly, requiring 15 months' time for normalization of size. Results of liver function tests, although improved, remained abnormal, and amyloid deposits remained in the marrow. A second patient also demonstrated dramatic diminution in proteinuria and improvement in liver function abnormalities, but macroglossia persisted. These observations suggest that amyloid organ involvement may be reversible with differences in organ responsiveness to chemotherapy. An empirical trial of chemotherapy may be indicated in some patients with progressive primary amyloidosis, and therapy may need to be continued for a prolonged period of time before seeing an effect.
两名患有进行性原发性淀粉样变性、单克隆血清和尿蛋白、多器官受累及肾病综合征的患者接受了美法仑和泼尼松治疗，为期一年。其中一名患者，脾肿大和肾病综合征对治疗迅速产生反应，但巨大肝肿大反应缓慢，需要15个月时间才能恢复正常大小。肝功能检查结果虽有改善，但仍不正常，骨髓中仍有淀粉样沉积物。第二名患者蛋白尿也显著减少，肝功能异常有所改善，但巨舌症持续存在。这些观察结果表明，淀粉样变器官受累可能是可逆的，不同器官对化疗的反应存在差异。对于一些进行性原发性淀粉样变性患者，可能需要进行化疗的经验性试验，并且可能需要持续治疗较长时间才能看到效果。
