Kappen J H, van Zaanen H C T, Snelder S M, van Tilburg A J P, Rudolphus A
Department of Pulmonology, Sint Franciscus Vlietland Groep, Rotterdam, The Netherlands.
Department of Haematology, Sint Franciscus Vlietland Groep, Rotterdam, The Netherlands.
BMJ Case Rep. 2017 Feb 6;2017:bcr2016218369. doi: 10.1136/bcr-2016-218369.
We present a rare case of grade II lymphomatoid granulomatosis (LYG) with pulmonary and gastrointestinal involvement. LYG is considered an Epstein-Barr virus-driven lymphoproliferative disorder that often presents with multiple nodular lesions in the lungs and sometimes involvement of skin and the central nervous system. Although the aetiology is unknown, it is associated with the use of immunosuppressives. Involvement of other organ systems is very rare. We successfully treated our patients with 6 cycles of R-CHOP and autologous stem cell transplantation with a major response at 20 months follow-up.
我们报告一例罕见的伴有肺部和胃肠道受累的II级淋巴瘤样肉芽肿病(LYG)。LYG被认为是一种由爱泼斯坦-巴尔病毒驱动的淋巴增殖性疾病,常表现为肺部多发结节性病变,有时累及皮肤和中枢神经系统。尽管病因不明,但它与免疫抑制剂的使用有关。其他器官系统受累非常罕见。我们用6个周期的R-CHOP方案和自体干细胞移植成功治疗了我们的患者,在20个月的随访中有显著反应。