CTICasesPulmonary Light-Chain Deposition Disease in Waldenstrom Macroglobulinemia
Imaging findings
A 67-year-old never-smoker referred for rheumatoid-arthritis-related lung disease has diffuse ground glass, severe bronchiectasis with strikingly diffuse thick bronchial wall thickening, and thin-walled cysts scattered in both lungs, progressing over five years to a mixed cystic and airway pattern. His rheumatoid factor was extremely elevated (9400 IU/mL) without clinical arthritis, reflecting a monoclonal IgM kappa, and bone marrow biopsy confirmed Waldenstrom macroglobulinemia.
Key takeaways
A markedly elevated rheumatoid factor without true arthritis should raise suspicion for a monoclonal IgM from a lymphoproliferative disorder, and diffuse thin-walled cysts with bronchial wall thickening in that setting suggest pulmonary light-chain deposition disease. The cystic spaces are consistent with immunoglobulin/protein deposition, an airway component (possibly follicular bronchiolitis) may coexist, and imaging (particularly the earlier cysts) can be the key clue prompting the correct hematologic diagnosis; tissue confirmation is often precluded by respiratory failure.
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