用利妥昔单抗治疗获得性因子 V 抑制剂。

IF 0.6 Q3 MEDICINE, GENERAL & INTERNAL BMJ Case Reports Pub Date : 2024-11-18 DOI:10.1136/bcr-2023-256475
Faisal Ansari, Yurhee Lee, Umar Ansari, Phyllis Kim
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引用次数: 0

摘要

获得性第五因子(FV)抑制剂极为罕见,其表现范围很广,从无症状的实验室异常到危及生命的严重出血。总体上的罕见性和临床表现的异质性给诊断带来了挑战。在这些情况下,目前还没有标准的免疫抑制疗法(IST)。文献中的大多数患者都接受了多种药物治疗,包括但不限于 IST 和/或重组产品的组合。在此,我们介绍了一例 50 多岁的男性患者,他最初表现为外周静脉和气管造口部位渗血,并伴有间歇性鼻衄。后来发现他的 FV 活性水平低于 1%,FV 抑制剂滴度为 184 贝塞斯达单位/毫升。患者最初使用新鲜冰冻血浆、血小板和氨甲环酸稳定病情,并静脉注射免疫球蛋白和糖皮质激素。然而,这只能使他的凝血功能得到轻微改善。随后,他接受了每周剂量的利妥昔单抗治疗,持续 4 周,并持续使用糖皮质激素,未出现并发症。关于利妥昔单抗可作为获得性 FV 抑制剂治疗方案的文献越来越多,这为该研究增添了新的内容。
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Acquired factor V inhibitor treated with rituximab.

Acquired factor V (FV) inhibitors are extremely rare and present with a broad spectrum ranging from asymptomatic laboratory anomalies to life-threatening critical bleeds. The overall rarity along with the heterogeneity of clinical presentations poses a challenge in diagnosis. There is currently no standard of care immunosuppressive therapy (IST) in these settings. Most patients in the literature receive multiple agents, including but not limited to combinations of IST and/or recombinant products.Here, we present a case of a man in his 50s who initially presented with oozing at peripheral IV and tracheostomy sites with intermittent epistaxis. He was later found to have an FV activity level of less than 1% and an FV inhibitor titre of 184 Bethesda units/mL. The patient was initially stabilised with fresh frozen plasma, platelets and tranexamic acid and treated with intravenous immunoglobulin and glucocorticoids. However, this resulted in only mild improvement in his coagulation studies. He was then treated with weekly doses of rituximab for 4 weeks with ongoing glucocorticoids without complications. This adds to the growing literature on rituximab as a possible treatment option for acquired FV inhibitors.

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来源期刊
BMJ Case Reports
BMJ Case Reports Medicine-Medicine (all)
CiteScore
1.40
自引率
0.00%
发文量
1588
期刊介绍: BMJ Case Reports is an important educational resource offering a high volume of cases in all disciplines so that healthcare professionals, researchers and others can easily find clinically important information on common and rare conditions. All articles are peer reviewed and copy edited before publication. BMJ Case Reports is not an edition or supplement of the BMJ.
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