Pulmonary Amyloidoma: A Rare Entity Complicating a Post-Heart Transplant Course

Abstract

<h3>Introduction</h3> Heart transplantation (HT) is a viable, life-prolonging therapy for patients with severe cardiac amyloidosis. Unique amyloid-related pathology may complicate their care. We present a case of a rare manifestation of amyloidosis complicating a post-HT course. <h3>Case Report</h3> A 51-year-old female with cardiac AL amyloidosis (treated with daratumumab, cyclophosphamide, bortezomib, and dexamethasone) and chronic kidney disease was transferred for advanced options evaluation. She presented in cardiogenic shock and PEA arrest requiring escalation to venoarterial-ECMO. Admission sputum culture was positive for <i>Enterobacter cloacae</i>. CT scan revealed right lower lobe consolidation with a 5cm cavitary lesion concerning for aspiration pneumonia. Broad spectrum antibiotics were initiated. Given near-complete resolution of her light chains on chemotherapy, Oncology agreed to proceed with transplantation. She was emergently listed as status 1 and underwent heart-kidney transplantation. One-week post-HT, she developed severe leukocytosis and abdominal discomfort but remained afebrile with only mild dyspnea. Abdominal CT incidentally noted an 11cm right lower lobe cavitary lung lesion. Dedicated CT chest raised concern for aspergilloma or other necrotizing pneumonia (Figure 1). Bronchoalveolar lavage had unremarkable fungal and viral studies but did reveal <i>Acinetobacter</i>. Antibiotics were appropriately adjusted. VATS was attempted but required conversion to thoracotomy with right lower lobectomy. Pathology revealed necroinflammatory findings and Congo-red stain-positive material both inside the cavity and extensively deposited within the cavity walls. These findings were consistent with pulmonary amyloidoma. <h3>Summary</h3> Though rare, pulmonary amyloidomas may be incidentally found as large cavitating lesions amongst patients with systemic amyloidosis. In the post-HT patient, they may serve as a nidus for infections and appear similar to necrotizing pneumonias, resulting in delays in diagnosis.