Hawken James, Knott Amy, Alsakkaf Wesam, Clark Amanda, Fayyaz Faisal
Department of Gastroenterology, Weston General Hospital, Weston-super-Mare, UK.
Department of Hepatology, University Hospitals Bristol NHS Foundation Trust, Bristol, UK.
Frontline Gastroenterol. 2019 Oct;10(4):434-437. doi: 10.1136/flgastro-2018-101116. Epub 2019 Jan 9.
Identification of acquired von Willebrand syndrome (AVWS) was key to treating a patient with chronic gastrointestinal (GI) bleeding due to angiodysplasia. After exhausting endoscopic and pharmacological options, the patient was successfully treated with rituximab. A 78-year-old man developed chronic GI bleeding from caecal and jejunal angiodysplasia. Red cell transfusion was required weekly despite argon plasma coagulation. A diagnosis of AVWS was made from analysis of clotting factors. Therapies including von Willebrand factor concentrate, thalidomide and tranexamic acid were unsuccessful. With failed endoscopic therapy and no viable surgical option, the patient was given intravenous immunoglobulins (IVIGs). Haemoglobin remained stable from this point. The impact on the patient and hospital of attending for IVIG every 3 weeks necessitated consideration to longer-term therapy. After a single course of rituximab, no further blood products, IVIG or rituximab were required. This case is the first to describe the use of rituximab in AVWS-associated angiodysplasia.
获得性血管性血友病综合征(AVWS)的诊断是治疗一名因血管发育异常导致慢性胃肠道(GI)出血患者的关键。在内镜和药物治疗手段均用尽后,该患者接受利妥昔单抗治疗获得成功。一名78岁男性因盲肠和空肠血管发育异常出现慢性GI出血。尽管进行了氩气血浆凝固治疗,仍需每周输注红细胞。通过凝血因子分析确诊为AVWS。包括血管性血友病因子浓缩物、沙利度胺和氨甲环酸在内的治疗均未成功。由于内镜治疗失败且没有可行的手术选择,患者接受了静脉注射免疫球蛋白(IVIG)。此后血红蛋白保持稳定。每3周进行一次IVIG治疗对患者和医院造成的影响使得有必要考虑长期治疗方案。在接受一个疗程的利妥昔单抗治疗后,患者不再需要进一步的血液制品、IVIG或利妥昔单抗。该病例是首例描述利妥昔单抗用于治疗与AVWS相关的血管发育异常的案例。