Balakrishnan Pradeep, Ing Matthew, Househ Zaid, Raguparan Ajantha
Department of Respiratory & Sleep Medicine St George Hospital Kogarah NSW Australia.
Department of Anatomical Pathology St George Hospital Kogarah NSW Australia.
Respirol Case Rep. 2021 May 26;9(7):e00789. doi: 10.1002/rcr2.789. eCollection 2021 Jul.
Pulmonary lymphomatoid granulomatosis (PLG) is a rare multisystem Epstein-Barr virus (EBV)-associated lymphoproliferative disorder. Exact incidence is unknown and, with its variable clinical presentation, making an accurate diagnosis of PLG can be difficult. We present two distinct cases at our tertiary centre that underline PLG's non-specific clinical presentations. This resulted in the failure of recognizing PLG early with consequently progressive fatal outcomes. The rationale is to enlighten us concisely the knowledge surrounding PLG and consider it as a potential differential diagnosis, particularly in those immunosuppressed patients with radiological evidence of worsening pulmonary infiltrates not responding to customary treatment for common diagnoses. Having a high degree of suspicion for PLG in the right setting and pursuing lung biopsy early if appropriate for histopathology examination would be justified. This is essential to correctly diagnose PLG up-front and subsequently utilize best management approach for a better survival and mortality risk outlook.
肺淋巴瘤样肉芽肿病(PLG)是一种罕见的多系统 Epstein-Barr 病毒(EBV)相关的淋巴增殖性疾病。确切发病率尚不清楚,且其临床表现多样,准确诊断 PLG 可能具有挑战性。我们在三级中心展示了两个不同的病例,突出了 PLG 的非特异性临床表现。这导致早期未能识别 PLG,从而导致病情逐渐发展至致命结局。目的是简明地启发我们了解围绕 PLG 的知识,并将其视为一种潜在的鉴别诊断,特别是在那些免疫抑制患者中,他们有肺部浸润恶化的影像学证据,对常见诊断的常规治疗无反应。在合适的情况下对 PLG 保持高度怀疑,并在适当时尽早进行肺活检以进行组织病理学检查是合理的。这对于预先正确诊断 PLG 并随后采用最佳管理方法以获得更好的生存和死亡率风险前景至关重要。