Zilko P J, Dawkins R L
Am J Med. 1975 Sep;59(3):448-52. doi: 10.1016/0002-9343(75)90406-4.
Described here is a 59 year old man with dermatomyositis and hypogammaglobulinemia. His muscle power improved after corticosteroid therapy, but extensive amyloidosis and repeated infections appeared. Bone marrow morphology suggested multiple myeloma, but treatment with cytotoxic drugs had no beneficial effect on the amyloidosis. Because of rapid progression of the amyloidosis and further infections, cytotoxic drug therapy was stopped, corticosteroid dosage was decreased, and supplementary immunoglobulin therapy was instituted. The infections occurred less frequently and the amyloidosis appeared to regress. This case suggests that immunosuppressive therapy may exacerbate amyloidosis. The literature is reviewed, and the possible role of humoral immunodeficiency in the pathogenesis of amyloidosis is discussed. It is suggested that supplementary immunoglobulin may be beneficial in amyloidosis.
本文报道了一名59岁患有皮肌炎和低丙种球蛋白血症的男性患者。皮质类固醇治疗后其肌肉力量有所改善，但出现了广泛的淀粉样变性和反复感染。骨髓形态学提示多发性骨髓瘤，但细胞毒性药物治疗对淀粉样变性并无有益效果。由于淀粉样变性迅速进展且感染进一步加重，停止了细胞毒性药物治疗，减少了皮质类固醇剂量，并开始了补充免疫球蛋白治疗。感染发生频率降低，淀粉样变性似乎有所消退。该病例提示免疫抑制治疗可能会加重淀粉样变性。本文对相关文献进行了综述，并讨论了体液免疫缺陷在淀粉样变性发病机制中的可能作用。建议补充免疫球蛋白可能对淀粉样变性有益。
