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A4131 - Cryptogenic Organizing Pneumonia as a Precursor of Primary Hodgkin's Disease: An Unreported Entity
Author Block: A. Lal1, J. Akhtar1, Y. Chen2, S. M. Davis3; 1Department of Medicine, Saint Vincent Hospital, Worcester, MA, United States, 2Department of Pathology, Saint Vincent Hospital, Worcester, MA, United States, 3St Vincent Hospital, Worcester, MA, United States.
Introduction: Association between Cryptogenic organizing pneumonia (COP) and underlying malignancies have been reported, mostly as a sequalae of pharmacological treatment or radiation therapy. Safadi et al, had described a case of BOOP preceding T cell rich B cell Non-Hodgkin’s lymphoma (NHL) 20 years ago. To the best of our knowledge and literature search, COP as a precursor for Hodgkin’s disease (HD) has never been reported before. Case: A 20-year-old female presented with complaints of hemoptysis and chest discomfort. CT chest showed a large 12 cm mass centered at the left mediastinum extending from the left lung apex with encasement of multiple vascular structures, highly suspicious for lymphoma or primary lung cancer. Patient underwent open lung biopsy (OLB) which surprisingly showed distal airspaces filled with macrophages and interlobular septa showing interstitial thickening and edema consistent with COP. She showed a prompt clinical and radiological response after initial treatment with steroid. Left lung mass reduced considerably in size after initial treatment. However after 4 months, patient started to have recurrence of symptoms. Repeat CT chest showed interval increase in size of left upper lobe mass with encasement of left bronchi and pulmonary arteries now even bigger than first presentation. In addition, there was subcarinal and hilar adenopathy. Repeat open lung biopsy showed lung parenchyma with a vaguely nodular proliferation with scattered admixed large atypical cells with irregular hyperchromatic nuclei, inclusion-like nucleoli consistent with Reed-Sternberg (RS) cells typical for classical Hodgkin lymphoma. PET scan showed markedly fluorodeoxyglucose (FDG) avid large left lobulated paramediastinal mass involving left upper lobe, left and right paratracheal, hilar and subcarinal lymph nodes. She was initiated on standard chemotherapy for HD in combination with radiation therapy resulting in remarkable reduction in size of mediastinal mass and pneumatocele formation at the site of left upper lobe mass. She has remained in remission for 7 years now. Discussion: This sequence of events is unfamiliar. COP diagnosed on biopsy in the early stages of the disease may be an inflammatory response to the presence of primary HD that was not diagnosed due to sample error, but response to steroid treatment goes against it. Second possibility to consider is that BOOP may have undergone a malignant transformation to a lymphoproliferative disorder with an unknown trigger. The symptom profile for COP and underlying malignancy may overlap, high index of suspicion is necessary and repeat OLB can be conclusive to guide the diagnosis and treatment.