Sugase Taro, Akimoto Tetsu, Kimura Takaaki, Yagisawa Takashi, Kusano Eiji, Nagata Daisuke
Division of Nephrology, Department of Internal Medicine, Jichi Medical University, Shimotsuke, Japan.
Division of Renal Surgery and Transplantation, Department of Urology, Jichi Medical University, Shimotsuke, Japan.
Clin Med Insights Case Rep. 2018 Mar 13;11:1179547618763371. doi: 10.1177/1179547618763371. eCollection 2018.
Acquired von Willebrand syndrome (AVWS) is a rare clinical entity presenting with heterogeneous hemorrhagic manifestations, although some subsets of patients with AVWS may be asymptomatic until they are exposed to major trauma, an invasive procedure, or surgery. We herein report one such case in a 73-year-old male patient with nephrotic syndrome with a prolonged active partial thromboplastin time. We initially did not deal with this distinct abnormal clotting profile seriously, but persistent bleeding after a retroperitoneoscopic-assisted renal biopsy that allowed us to ascribe his nephrotic syndrome to membranous nephropathy fortuitously led to the discovery of concurrent AVWS. We feel that an accurate and prompt diagnosis as well as awareness of the disease remain a challenge for physicians and therefore strongly recommend the further accumulation of experiences similar to our own in a prospective manner. This report underscores the pitfalls associated with determining the bleeding risk, including an insufficient assessment and improper weighting of an abnormal clotting profile prior to the invasive procedure. Several management concerns that emerged in the current case are also discussed.
获得性血管性血友病综合征(AVWS)是一种罕见的临床病症,表现为异质性出血症状,不过部分AVWS患者在遭受重大创伤、接受侵入性操作或手术之前可能没有症状。我们在此报告一例73岁男性肾病综合征患者,其活化部分凝血活酶时间延长。最初我们并未重视这一明显异常的凝血指标,但在逆行腹腔镜辅助肾活检后持续出血,这使我们偶然将其肾病综合征归因于膜性肾病,进而发现了并发的AVWS。我们认为,准确、及时的诊断以及对该疾病的认识对医生而言仍是一项挑战,因此强烈建议以前瞻性方式进一步积累类似我们自己的经验。本报告强调了确定出血风险时存在的陷阱,包括在侵入性操作前对异常凝血指标评估不足和权重不当。文中还讨论了当前病例中出现的几个管理方面的问题。