le Quellec A, Sotto A, Ciurana A J
Department of Internal Medicine A, Saint-Eloi Hospital, Montpellier, France.
J Intern Med. 1993 Sep;234(3):329-30. doi: 10.1111/j.1365-2796.1993.tb00751.x.
We describe the case of a 51-year-old man with systemic amyloidosis in whom factor X activity was initially 6% of the normal. Amyloidosis was responsible for congestive heart failure and a nephrotic syndrome but there was no bleeding diathesis. A 12-month trial of melphalan and prednisone failed to improve cardiac and renal dysfunction; factor X levels remained low. Eighteen months after this treatment was stopped, factor X spontaneously normalized although renal insufficiency persisted. We suggest that the possibility of a spontaneous factor X recovery must be considered when evaluating efficacy of therapeutic agents in amyloidosis.
我们描述了一例51岁患有系统性淀粉样变性的男性患者，其最初的凝血因子X活性为正常水平的6%。淀粉样变性导致了充血性心力衰竭和肾病综合征，但不存在出血倾向。进行了为期12个月的美法仑和泼尼松试验，未能改善心脏和肾功能障碍；凝血因子X水平仍维持在低水平。在停止该治疗18个月后，尽管肾功能不全仍然存在，但凝血因子X自发恢复正常。我们建议，在评估治疗药物对淀粉样变性的疗效时，必须考虑凝血因子X自发恢复正常的可能性。
