原发性肺霍奇金淋巴瘤(PPHL)是一种罕见的淋巴瘤亚型,占原发性肺淋巴瘤的一小部分。由于其罕见和非特异性症状,PPHL经常提出诊断挑战。本病例报告介绍了一例PPHL模拟肉芽肿病伴多血管炎的独特病例。强调诊断过程中遇到的困难。一名53岁的女性表现出模糊的症状,包括虚弱,水肿,干咳,鼻腔溃疡.实验室调查显示C反应蛋白水平升高,嗜中性粒细胞增多症的白细胞计数,和淋巴细胞减少症.对于可疑的多血管炎,口服皮质类固醇的初始治疗没有反应。患者随后出现低热和瘙痒性红斑皮疹。诊断程序,包括支气管刷检,支气管冲洗,纵隔淋巴结活检,鼻腔溃疡活检,和最初的肺活检,尚无定论,并导致排除肉芽肿合并多血管炎。随后的计算机断层扫描显示左肺疾病进展。肺活检显示纤维化组织,结节含有霍奇金-里德-斯特恩伯格细胞,导致经典霍奇金淋巴瘤的最终诊断,结节性硬化症亚型。正电子发射断层扫描扫描结果证实了PPHL。患者接受多种化疗方案,与伦妥昔单抗vedotin证明疗效作为唯一有效的治疗方法。这个特殊的PPHL病例强调了涉及多学科临床医生团队的广泛诊断和治疗检查。放射科医生,和病理学家。提高对PPHL及其独特特征的认识将有助于将来对类似病例的诊断,受益于临床医生和病理学家。
Primary pulmonary Hodgkin\'s lymphoma (PPHL) is a rare subtype of lymphoma that comprises a small percentage of primary pulmonary lymphomas. Due to its rarity and nonspecific symptoms, PPHL often presents diagnostic challenges. This
case report presents a unique
case of PPHL mimicking granulomatosis with polyangiitis, emphasizing the difficulties encountered during the diagnostic process. A 53-year-old female presented with vague symptoms including weakness, oedema, dry cough, and nasal cavity ulceration. Laboratory investigations revealed elevated C-reactive protein levels, a white blood cell count with neutrophilia, and lymphopaenia. Initial treatment with oral corticosteroids for suspected polyangiitis yielded no response. The patient subsequently developed a low-grade fever and pruritic erythematous rash. Diagnostic procedures, including bronchial brush biopsy, bronchial washing, mediastinal lymph node biopsy, nasal cavity ulceration biopsy, and initial lung biopsy, were inconclusive and resulted in exclusion of granulomatosis with polyangiitis. A subsequent computed tomography scan indicated disease progression in the left lung. A lung biopsy revealed fibrotic tissue with nodules containing Hodgkin- Reed-Sternberg cells, leading to the final diagnosis of classic Hodgkin lymphoma, nodular sclerosis subtype. Positron emission tomography scan findings confirmed PPHL. The patient received multiple chemotherapeutic regimens, with brentuximab vedotin demonstrating efficacy as the sole effective treatment. This exceptional
case of PPHL underscores the extensive diagnostic and therapeutic workup involving a multidisciplinary team of clinicians, radiologists, and pathologists. Increased awareness of PPHL and its distinctive features will aid in the diagnosis of similar cases in the future, benefitting both clinicians and pathologists.