Department of Pulmonology, University Hospitals Leuven , Leuven, Belgium.
Department of Pulmonology, AZ Delta Roeselare-Menen-Torhout , Belgium.
Acta Clin Belg. 2020 Oct;75(5):370-374. doi: 10.1080/17843286.2019.1655232. Epub 2019 Aug 17.
Pulmonary lymphomatoid granulomatosis (PLG) is a rare angiocentric and angiodestructive EBV-associated lymphoproliferative disorder which almost always affects the lungs. PLG is more commonly diagnosed in patients with immunodeficiency and is associated with Epstein-Barr virus (EBV). 'Drug induced PLG' or 'iatrogenic immunodeficiency-associated lymphoproliferative disorder' is a special form of PLG described in patient with inflammatory bowel diseases treated with Azathioprine.
We report a case of drug-induced PLG in a 68-year-old patient with Crohn's disease presenting with pain at the right hemithorax, fatigue and shortness of breath with a pulmonary mass.
Although initial diagnostic findings were misleading, an open lung biopsy eventually led to the diagnosis of drug-induced PLG.
The diagnosis of PLG is challenging because the disease is rare and the histological features can be very subtle. Correct diagnosis relies on histopathology and immunohistochemical staining and EBV RNA in situ hybridization with sampling of large and different amounts of pathologic tissue in the hands of expert pathologists. In drug-induced PLG specifically, withdrawal of the immunosuppressive agent can lead to disease regression.
肺淋巴瘤样肉芽肿病(PLG)是一种罕见的血管中心性和血管破坏性 EBV 相关淋巴增生性疾病,几乎总是影响肺部。PLG 在免疫功能低下的患者中更为常见,与 Epstein-Barr 病毒(EBV)有关。“药物诱导的 PLG”或“医源性免疫缺陷相关淋巴增生性疾病”是在接受硫唑嘌呤治疗的炎症性肠病患者中描述的 PLG 的一种特殊形式。
我们报告了一例 68 岁克罗恩病患者发生药物诱导的 PLG 的病例,该患者表现为右侧胸痛、乏力和呼吸急促,并伴有肺部肿块。
尽管最初的诊断结果具有误导性,但开胸肺活检最终导致了药物诱导的 PLG 的诊断。
PLG 的诊断具有挑战性,因为该病罕见,组织学特征可能非常细微。正确的诊断依赖于组织病理学和免疫组织化学染色以及 EBV RNA 原位杂交,并由专家病理学家采集大量不同数量的病理组织样本。具体来说,在药物诱导的 PLG 中,停用免疫抑制剂可导致疾病消退。