CRA51 INTO THE UNKNOWN – A RARE CASE OF PRIMARY PULMONARY HODGKIN LYMPHOMA (PPHL)

Syahrinnaquiah Samsuddin1, Yen Shen Wong1, Am Basheeri Alias1, Mohd Zhafran Zainal1, Aisya Natasya Musa1

1 Faculty of Medicine, UITM Sungai Buloh

Introduction
Primary pulmonary lymphoma is a rare disorder, accounting for only 0.3% of all primary pulmonary malignancies. Among these, PPHL is exceptionally uncommon, with only sporadic cases reported in the literature. We present a case of PPHL diagnosed through histopathological analysis and confirmed with PET scans.

Case Report
A 45-year-old woman, a non-smoker with no significant medical or family history, presented with a two-week history of cough and occasional hemoptysis. She denied fever, night sweats, or weight loss. Initial chest radiograph showed a right upper lobe mass. Bronchoscopy was non-diagnostic, and a CT-guided biopsy was suspicious of interstitial lung disease. She underwent radial EBUS-guided forceps biopsy under fluoroscopic guidance revealed classical Hodgkin lymphoma. A PET-CT scan demonstrated disease confined to the right upper lobe without hilar or mediastinal lymphadenopathy, confirming primary pulmonary origin. She was subsequently referred to hematology team for definitive treatment

Discussion
The diagnosis of PPHL is difficult but according to diagnostic criteria proposed by Kert et al., PPHL is defined by: (1) histopathological confirmation of classical Hodgkin lymphoma, (2) disease localized to the lung without evidence of extrapulmonary or nodal involvement, and (3) exclusion of other primary causes and our case met all these criteria. To date, there are no established treatment guidelines but a combination of chemotherapy with or without involved-field radiotherapy has shown favorable outcomes. In selected cases, surgical resection may be curative, particularly when the lesion is localized.

Conclusion
PPHL is a rare but important differential diagnosis for solitary pulmonary lesions.  Accurate diagnosis hinges on a combination of clinical suspicion, radiologic assessment, and confirmatory histopathology.

Keywords: primary pulmonary Hodgkin lymphoma, lymphoma