Chkheidze Rati, Joseph Ranjit, Burner James, Matevosyan Karen
Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX, 75390.
J Clin Apher. 2018 Jun;33(3):412-418. doi: 10.1002/jca.21573. Epub 2017 Aug 9.
Intractable pruritus of cholestasis leads to significant morbidity. Therapeutic plasma exchange (TPE) has been shown to be an effective alternative in the setting of refractory pruritus associated with cholestatic liver disease based on several individual reports. Due to rarity of this approach to intractable pruritus, the literature is sparse and therefore TPE, as a treatment for refractory pruritus is currently not in the apheresis guidelines. We present three additional patients with severe intractable pruritus of cholestasis successfully treated with plasma exchange to add to the mounting literature showing this as an effective and safe adjunctive therapy.
Three patients underwent serial plasma exchange procedures to control pruritus. Frequency of plasma exchange was three times a week, with slow taper upon improvement of pruritus. Total bile acid levels were assessed before procedures.
All three patients had an intractable pruritus with different underlying etiologies of cholestasis. All three patients showed significant improvement in pruritus, with none or minimal pruritus in one patient with primary biliary cirrhosis. Pre procedure bile acids levels were decreased initially, but showed rebound increase upon tapering of plasma exchange, without increased pruritus. No serious side effects or complications were observed.
Our results in conjunction with the published literature show that severe and intractable pruritus associated with cholestasis could be successfully treated with TPE, irrespective of the underlying disease, and can be done safely.
胆汁淤积性顽固性瘙痒会导致严重的发病率。基于一些个案报道,治疗性血浆置换(TPE)已被证明是治疗与胆汁淤积性肝病相关的难治性瘙痒的一种有效替代方法。由于这种治疗顽固性瘙痒的方法罕见,相关文献稀少,因此TPE作为难治性瘙痒的一种治疗方法目前未被纳入血液分离指南。我们报告另外3例胆汁淤积性严重顽固性瘙痒患者通过血浆置换成功治疗,以补充越来越多表明这是一种有效且安全的辅助治疗方法的文献。
3例患者接受了系列血浆置换程序以控制瘙痒。血浆置换频率为每周3次,瘙痒改善后逐渐减量。在进行血浆置换前评估总胆汁酸水平。
所有3例患者均有顽固性瘙痒,胆汁淤积的潜在病因各不相同。所有3例患者的瘙痒均有显著改善,1例原发性胆汁性肝硬化患者无瘙痒或仅有轻微瘙痒。血浆置换前胆汁酸水平最初下降,但在血浆置换减量时出现反弹升高,且瘙痒未加重。未观察到严重副作用或并发症。
我们的结果与已发表的文献表明,与胆汁淤积相关的严重顽固性瘙痒可以通过TPE成功治疗,无论潜在疾病如何,并且可以安全进行。