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Use of PEG-asparaginase in monomorphic epitheliotropic intestinal T-cell lymphoma a disease with diagnostic and therapeutic challenges

机译:PEG-天冬酰胺酶在单形上皮性肠道T细胞淋巴瘤中的应用这种疾病具有诊断和治疗挑战

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摘要

Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), previously known as enteropathy associated T-cell lymphoma (EATL) type II, is a rare haematological malignancy with a difficult and delayed diagnosis. Symptoms can include abdominal pain, weight loss, and chronic diarrhoea. However, most patients are only diagnosed after complications, such as perforation or obstruction, have developed. There is no standard treatment for MEITL; most accepted regimens consist of surgical resection and anthracycline-based chemotherapy. Prognosis is poor with an approximate survival of less than a year. Even though other therapies, such as autologous stem cell transplant, has shown promising results, not all patients can tolerate this course of treatment especially if they are elderly, have several comorbidities or are malnourished. Innovative therapies that improve survival and can be used as an alternative for more intensive treatment are needed. We report the use of PEG-asparaginase along with conventional anthracycline therapy in a 70-year-old woman diagnosed with MEITL, who went into remission and survived for more than one year before succumbing to relapsed disease.
机译:单形上皮性肠道T细胞淋巴瘤(MEITL),以前称为II型肠病相关性T细胞淋巴瘤(EATL),是一种罕见的血液系统恶性肿瘤,诊断困难且延迟。症状可能包括腹痛,体重减轻和慢性腹泻。但是,大多数患者只有在出现诸如穿孔或阻塞之类的并发症后才被诊断出。 MEITL没有标准治疗方法;大多数被接受的治疗方案包括手术切除和基于蒽环类的化疗。预后较差,生存期不到一年。尽管其他疗法(例如自体干细胞移植)已显示出令人鼓舞的结果,但并非所有患者都可以忍受这一治疗过程,尤其是在年老,患有多种合并症或营养不良的患者中。需要创新的疗法来提高生存率,并可以用作更深入的治疗的替代方法。我们报告了在一名确诊为MEITL的70岁女性中,将PEG-天冬酰胺酶与常规蒽环类疗法一起使用,该女性进入缓解期并存活了一年以上,之后死于复发性疾病。

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