Alsultan Mohammad, Jomaa Hiba, Shukri Arwa, Hajij Fatima, Hassan Qussai
Department of Nephrology.
Department of Hematology.
Ann Med Surg (Lond). 2023 Apr 3;85(5):1848-1851. doi: 10.1097/MS9.0000000000000353. eCollection 2023 May.
Acquired hemophilia refers to the development of a clotting factor deficiency typically, when autoantibodies are produced against coagulation factor and tend to be in the elderly and rare in children.
A 12-year-old girl with steroid-resistant nephrosis (SRN) was admitted complaining of pain in her right leg and an ultrasound showed a hematoma in her right calf. The coagulation profile revealed partial thromboplastin time prolongation and high titers (156 BU) of anti-factor VIII inhibitors were observed. Where half of the patients with antifactor VIII inhibitors were associated with underlying disorders, additional tests were performed that rule out secondary causes. This patient with long-standing SRN, who was on a maintenance dose of prednisone for six years, was complicated with acquired hemophilia A (AHA). In different to the last recommendations of AHA treatment, we preferred using cyclosporine; which is considered the initial second-line therapy for children with SRN. Complete remission was achieved of both disorders after a month with no recurrence of nephrosis or bleeding events.
To our knowledge, nephrotic syndrome with AHA was reported in only three patients, two cases after remission and one during a relapse but no one were treated with cyclosporine. The authors encountered the first case of cyclosporine treatment for AHA in a patient with SRN. This study supports the use of cyclosporine to treat AHA, especially with nephrosis.
获得性血友病通常是指在产生针对凝血因子的自身抗体时出现凝血因子缺乏，多见于老年人，儿童罕见。
一名12岁患类固醇抵抗性肾病（SRN）的女孩因右腿疼痛入院，超声检查显示右小腿有血肿。凝血检查显示部分凝血活酶时间延长，且观察到抗凝血因子VIII抑制剂的高滴度（156 BU）。由于半数抗凝血因子VIII抑制剂患者与潜在疾病有关，因此进行了其他检查以排除继发原因。该长期患有SRN且已接受泼尼松维持剂量治疗六年的患者并发了获得性血友病A（A（AHA）。与AHA治疗的最新建议不同，我们更倾向于使用环孢素；环孢素被认为是儿童SRN的初始二线治疗药物。一个月后两种疾病均完全缓解，肾病或出血事件均未复发。
据我们所知，仅报道了3例患有AHA的肾病综合征患者，2例在缓解后，1例在复发期间，但均未用环孢素治疗。作者遇到了首例用环孢素治疗SRN患者AHA的病例。本研究支持使用环孢素治疗AHA，尤其是合并肾病时。
