An Uncommon Hematological Cause of Interstitial Lung Disease

Document Type

Conference Proceeding

Publication Date

5-2025

Publication Title

American Journal of Respiratory and Critical Care Medicine

Abstract

Interstitial lung diseases (ILD) are a group of conditions that are characterized by inflammation or fibrosis of the pulmonary parenchyma. They represent a diagnostic dilemma for the clinician as ILD can be caused by different etiologies including infectious, inflammatory, and environmental causes. In some instances, ILD are idiopathic. Here we describe a case of a hematologic condition that presented as ILD. An 83-year-old woman presented due to dyspnea and cough. She reported 15-pound weight loss over the preceding six months. Her medical history included hypertension, hyperlipidemia, osteoporosis. Review of systems was negative for arthralgia, skin rash, diarrhea, or vision abnormalities. She reported a 20-pack-year history of smoking and quit approximately 40 years ago. She lived in a 100-year-old house with mold in the basement but had no other history of occupational exposures. Blood work revealed lymphocytosis, and peripheral flow cytometry was consistent with B-cell lymphoproliferative disorder. A high-resolution chest CT revealed moderate, peripheral, lower-lobe predominant interstitial opacities with prominent consolidation compatible with a non-UIP type pattern. It showed nodular densities up to 10 mm on the left side (see image A). A pulmonary function test indicated a moderate restrictive lung disease pattern with severe diffusion impairment. Given her persentation and work-up, a CT-guided lung biopsy was performed, which was positive for and disclosed pulmonary involvement of B-cell non-Hodgkin lymphoma, compatible with extranodal marginal zone B-cell lymphoma (see image B and C). There was no overt evidence of carcinoma. The patient was given weekly dose of rituximab for four doses. She reported better breathing, her hypoxia resolved, and she no longer required supplemental oxygen. Extranodal marginal zone B‐cell lymphoma of mucosa associated lymphoid tissue (MALT), also known as MALT lymphoma, comprises up to 8% of all non‐Hodgkin lymphomas (NHL). MALT lymphoma originating in the lung, also known as pulmonary marginal zone lymphoma (PMZL), is a rare disease representing up to 14% of MALT lymphomas; however, it is the most common NHL affecting the lung. The most frequent radiographic patterns of PMZL are consolidation, nodular disease and mass, like our patient presentation. An adequate history taking looking for the presence of B symptoms and prior history of hematologic malignancy should raise the concern for PMZL as a possible etiology in patients that present with respiratory symptoms and abnormal chest imaging with lung nodules and consolidation and should trigger prompt tissue sampling to confirm diagnosis and start treatment as soon as feasible.

Volume

211

First Page

A6995

Comments

American Thoracic Society (ATS) International Conference, May 16-21, 2025, San Francisco, CA

Last Page

A6995

DOI

10.1164/ajrccm.2025.211.Abstracts.A6995

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