Bednarek-Skublewska Anna, Majdan Maria, Targońska-Stepniak Bozena, Ksiazek Andrzej
Katedra i Klinika Nefrologii Akademii Medycznej w Lublinie im. prof. Feliksa Skubiszewskiego.
Pol Arch Med Wewn. 2006 Sep;116(3):868-72.
Amyloidosis is a general term encompassing a group of disorders morphologically characterized by a deposition of proteinaceous fibrillar material in the tissues. The secondary amyloidosis is associated with systemic inflammatory processes, chronic infections or neoplasm. These processes are associated with elevated levels of circulating serum amyloid protein A (SAA). We have described a unique case of led to systemic amyloidosis associated with tumor of urinary bladder with significant renal involvement and acquired disorders of blood coagulation factor IX and X deficiency. A renal biopsy specimen with Congo red stain showed diffuse deposits of amyloidosis. Immunohistochemical examination was not done. Factor IX and X was determinated and to be 10-16% of normal values. Timely patient has failed to response to fresh frozen plasma and vitamin K and favorable responded to once weekly administrated factor IX and X concentrate. Progressive deterioration of renal function required hemodialysis, but he died one year later.
淀粉样变性是一个通用术语，涵盖了一组疾病，其形态学特征是在组织中沉积蛋白质纤维状物质。继发性淀粉样变性与全身性炎症过程、慢性感染或肿瘤有关。这些过程与循环血清淀粉样蛋白A（SAA）水平升高有关。我们描述了一例独特的病例，该病例导致系统性淀粉样变性与膀胱肿瘤相关，并伴有严重的肾脏受累以及获得性凝血因子IX和X缺乏症。经刚果红染色的肾脏活检标本显示淀粉样变性的弥漫性沉积。未进行免疫组织化学检查。测定凝血因子IX和X为正常值的10 - 16%。及时给予新鲜冰冻血浆和维生素K治疗，患者无反应，而每周一次给予凝血因子IX和X浓缩剂则有良好反应。肾功能进行性恶化需要血液透析，但患者一年后死亡。
