Nodular pulmonary amyloidosis presenting as a localized manifestation of lymphoproliferative disease: A three-case series.

Pulmonary amyloidosis presents in nodular, tracheobronchial, and diffuse alveolar-septal forms. Nodular pulmonary amyloidosis (NPA) is usually localized and asymptomatic. It is often linked to indolent B-cell lymphoproliferative disorders (LPD). The National Comprehensive Cancer Network recommends long-term follow-up and evaluation for low-grade B-cell lymphoproliferative disorders when specific clinical features are present. However, there are no established screening guidelines specifically for detecting lymphoma in patients with nodular pulmonary amyloidosis. We describe three patients with NPA who later developed LPD. A 53-year-old woman with biopsy-proven κ-type localized AL amyloidosis was later found to have monoclonal gammopathy of undetermined significance. A 63-year-old man with prior hairy cell leukemia had λ-type NPA and subsequently Epstein-Barr virus-positive Hodgkin lymphoma. A 74-year-old man undergoing pneumothorax surgery had NPA with bronchus-associated lymphoid tissue lymphoma and later gastric mucosa-associated lymphoid tissue lymphoma. None of them had systemic amyloidosis at diagnosis. These cases suggest NPA may coexist with or precede LPD, underscoring the importance of longitudinal monitoring. Larger studies are needed to help standardize screening, as early identification of an underlying LPD could potentially improve hematologic outcomes.