Saini Tanjot S, Dass Sharad K, Dhillon Simran
Internal Medicine, University of California San Francisco, Fresno, USA.
Internal Medicine, O'Connor Hospital, San Jose, USA.
Cureus. 2022 Feb 17;14(2):e22324. doi: 10.7759/cureus.22324. eCollection 2022 Feb.
Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial lung disease that affects young adults and is typically misdiagnosed or presents concurrently with more common respiratory conditions such as asthma or chronic obstructive lung disease (COPD). A combination of imaging, tissue biopsy, and clinical presentation is used for diagnosis since no definitive criteria have been established. Current standards are based on vague descriptors such as clustering of histologic markers but lack quantitative analysis. In this case report, we present a patient who was initially diagnosed with PLCH, but the lack of unanimity for diagnostic requirements led to conflicting diagnoses between institutions which may have prevented optimal care for the patient. The disparity limited new, alternative therapies for our patient that may have been beneficial since he was clinically not improving with smoking cessation and standard of care for obstructive lung diseases. However, quantitative endorsements for tissue analysis, such as requiring more than 30 Cd-1a stained Langerhans cells per high power field (HPF), may reduce discrepancies associated with current techniques. It is imperative that clear standards are established due to the unique treatment these patients require that is atypical from other pulmonary diseases such as asthma and COPD. New chemotherapeutic regimens such as cladribine and vemurafenib require oncologic care and have more broad side effects than typical pulmonary treatments, which emphasize the need for accurate diagnoses before starting treatment. Existing standards have created circumstances where PLCH is a differential but cannot be ruled out due to unclear criteria and limited research.
肺朗格汉斯细胞组织细胞增多症（PLCH）是一种罕见的间质性肺疾病，主要影响年轻人，通常被误诊，或与哮喘或慢性阻塞性肺疾病（COPD）等更常见的呼吸道疾病同时出现。由于尚未确立明确的诊断标准，因此结合影像学、组织活检和临床表现进行诊断。目前的标准基于诸如组织学标志物聚集等模糊描述，但缺乏定量分析。在本病例报告中，我们介绍了一名最初被诊断为PLCH的患者，但由于诊断要求缺乏一致性，导致不同机构之间的诊断存在冲突，这可能阻碍了对患者的最佳治疗。这种差异限制了为我们的患者提供可能有益的新的替代疗法，因为他在戒烟和阻塞性肺病的标准治疗下临床症状并未改善。然而，对组织分析的定量认可，例如要求每个高倍视野（HPF）有超过30个Cd-1a染色的朗格汉斯细胞，可能会减少与当前技术相关的差异。由于这些患者需要独特的治疗，与哮喘和COPD等其他肺部疾病不同，因此必须建立明确的标准。新的化疗方案，如克拉屈滨和维莫非尼需要肿瘤治疗，并且比典型的肺部治疗有更广泛的副作用，这强调了在开始治疗前进行准确诊断的必要性。现有标准造成了这样的情况，即PLCH是一个鉴别诊断，但由于标准不明确和研究有限而无法排除。
