Carvão Joana, Magno Pereira Vítor, Jacinto Fernando, Sousa Andrade Carla, Jasmins Luís
Gastroenterology Department, Hospital Central do Funchal, Funchal, Portugal.
Hemato-Oncology Department, Hospital Central do Funchal, Funchal, Portugal.
GE Port J Gastroenterol. 2019 Jul;26(4):279-284. doi: 10.1159/000493350. Epub 2018 Nov 28.
Hepatosplenic T-cell lymphoma (HSTCL) is an extremely rare and aggressive form of non-Hodgkin lymphoma associated with poor response to treatment and high mortality. There is an increased incidence among patients with inflammatory bowel disease, especially young male patients under 35 years old and on combination therapy (thiopurine and anti-TNF-α). We describe a case of HSTCL in a young male patient with stenosing ileal Crohn's disease on azathioprine monotherapy for 4.8 years admitted to our hospital with intra- abdominal sepsis. Despite chemotherapy, the patient eventually died 1 month after the diagnosis. Through a literature review, we identified 18 additional cases of HSTCL in Crohn's disease patients that had only been treated with thiopurine monotherapy. The authors intend to highlight the rarity of this diagnosis especially with azathioprine monotherapy and the diagnostic challenge in a case that presented with intra-abdominal sepsis.
肝脾T细胞淋巴瘤（HSTCL）是一种极其罕见且侵袭性强的非霍奇金淋巴瘤，与治疗反应不佳和高死亡率相关。炎症性肠病患者的发病率有所增加，尤其是35岁以下接受联合治疗（硫嘌呤和抗TNF-α）的年轻男性患者。我们描述了一例患有狭窄性回肠克罗恩病的年轻男性患者，该患者接受硫唑嘌呤单药治疗4.8年，因腹腔内脓毒症入住我院，被诊断为HSTCL。尽管进行了化疗，患者最终在诊断后1个月死亡。通过文献回顾，我们又发现了18例仅接受硫嘌呤单药治疗的克罗恩病患者发生HSTCL的病例。作者旨在强调这种诊断的罕见性，尤其是在硫唑嘌呤单药治疗的情况下，以及在出现腹腔内脓毒症的病例中的诊断挑战。
