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目的探讨原发性淀粉样变并发凝血因子Ⅶ缺乏的临床特征、发病机制及治疗方法。方法对1例原发性淀粉样变并发凝血因子Ⅶ缺乏的患者进行病史采集、体格检查和实验室检查,并行组织学活检,结合文献分析其发病机制,探讨治疗方法。结果该患者有自发性肌肉出血、皮肤黏膜出血、上消化道出血,肝脾肿大,凝血酶原时间(PT)、活化部分凝血酶原时间(APTT)延长,凝血因子检查Ⅶ因子活性明显降低,组织活检证实为淀粉样变性。结论淀粉样变并发凝血因子Ⅶ缺乏临床上少见,国内外罕见报道,目前认为原发性淀粉样变性并发凝血因子缺乏的机制是凝血因子与淀粉样变纤维结合,沉积在组织中。本病以化疗为主,远期疗效差。有严重出血或需行手术患者可予输注凝血因子治疗。
Objective To investigate the clinical features, pathogenesis and treatment of primary amyloidosis complicated with coagulation factor Ⅶ deficiency. Methods One patient with primary amyloidosis complicated with coagulation factor Ⅶ was collected for medical history, physical examination and laboratory examination. The biopsy was performed in combination with the literature to analyze its pathogenesis and explore the treatment methods. Results The patient had spontaneous muscular hemorrhage, mucocutaneous bleeding, upper gastrointestinal bleeding, hepatosplenomegaly, prothrombin time (PT), partial prothrombin time (APTT) prolonged, and the activity of factor VII in coagulation factor test was significantly lower , Biopsy confirmed as amyloidosis. Conclusions Amyloidosis complicated with coagulation factor Ⅶ deficiency is clinically rare. It is rarely reported at home and abroad. It is currently thought that the mechanism of primary amyloidosis complicated with coagulation factor deficiency is the combination of coagulation factors and amyloid fibrils deposited in tissues. Chemotherapy-based disease, long-term poor efficacy. Severe bleeding or surgery may require transfusion coagulation factor therapy.