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

## TL;DR

This case series shows that nodular pulmonary amyloidosis can be linked to or precede B-cell lymphoproliferative disorders, highlighting the need for long-term monitoring.

## Contribution

The study presents three cases where nodular pulmonary amyloidosis was followed by lymphoproliferative disease, suggesting a potential clinical link.

## Key findings

- Nodular pulmonary amyloidosis may coexist with or precede lymphoproliferative disorders.
- Surgical biopsy is often needed to detect underlying lymphoid proliferation masked by amyloid deposits.
- Long-term monitoring is recommended to detect potential hematologic disorders early.

## Abstract

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.

•Nodular pulmonary amyloidosis is often incidental and may precede indolent B-cell lymphoproliferative disorders.•Accurate diagnosis may require surgical biopsy due to amyloid deposits masking underlying lymphoid proliferation.•Our case series emphasizes the need for longitudinal follow-up and structured surveillance for early hematologic diagnosis.

Nodular pulmonary amyloidosis is often incidental and may precede indolent B-cell lymphoproliferative disorders.

Accurate diagnosis may require surgical biopsy due to amyloid deposits masking underlying lymphoid proliferation.

Our case series emphasizes the need for longitudinal follow-up and structured surveillance for early hematologic diagnosis.