Sławiński Lucjan, Sołek Julia Maria, Miłkowska-Dymanowska Joanna, Jesionek-Kupnicka Dorota, Góra-Tybor Joanna, Mikulski Damian, Braun Marcin
Department of Pathology, Chair of Oncology, Medical University of Łódź, Łódź, Poland.
Department of Pneumology, 2 Chair of Internal Medicine, Medical University of Łódź, Łódź, Poland.
Contemp Oncol (Pozn). 2023;27(2):113-117. doi: 10.5114/wo.2023.131034. Epub 2023 Aug 20.
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.
原发性肺霍奇金淋巴瘤（PPHL）是淋巴瘤的一种罕见亚型，在原发性肺淋巴瘤中占比很小。由于其罕见性和非特异性症状，PPHL常常带来诊断挑战。本病例报告呈现了一例独特的PPHL，其表现类似肉芽肿性多血管炎，强调了诊断过程中遇到的困难。一名53岁女性出现了包括乏力、水肿、干咳和鼻腔溃疡等模糊症状。实验室检查显示C反应蛋白水平升高、白细胞计数伴中性粒细胞增多以及淋巴细胞减少。最初因疑似多血管炎给予口服糖皮质激素治疗无效。患者随后出现低热和瘙痒性红斑皮疹。包括支气管刷检、支气管灌洗、纵隔淋巴结活检、鼻腔溃疡活检和初次肺活检在内的诊断程序均无定论，排除了肉芽肿性多血管炎。随后的计算机断层扫描显示左肺病情进展。肺活检显示纤维化组织中有含霍奇金-里德-斯腾伯格细胞的结节，最终诊断为经典型霍奇金淋巴瘤，结节硬化亚型。正电子发射断层扫描结果证实为PPHL。患者接受了多种化疗方案，其中维布妥昔单抗显示出作为唯一有效治疗的疗效。这例特殊的PPHL病例强调了由临床医生、放射科医生和病理科医生组成的多学科团队所进行的广泛诊断和治疗评估。提高对PPHL及其独特特征的认识将有助于未来对类似病例的诊断，使临床医生和病理科医生都受益。
