Аннотация:Her complete blood count was normal although ESR was 91mm/hr.HRCT showed persisting diffuse ground glass infiltrates throughout the lungs with a background of emphysema sparing extreme apices bases and sub-pleural regions with mediastinal and right hilar adenopathy.EBUS guided biopsy showed macrophages, multi-nucleated giant cells, other blood constituent cells and bland squamous cells without abnormal cells and congo red staining showed apple green bifringents.As she was having recurrent infections bloods were analyzed for immunoglobulin levels which showed a 22.8 pg/L of Para protein levels with elevated lambda light chains (143mg/L) with a kappa/Lambda ratio of 0.08.Her bone marrow analysis showed 6.9% of plasma cells.A diagnosis of IgG lambda myeloma was made and skeletal survey did not show lytic lesions.With the diagnosis of lung amyloidosis with IgG lambda myeloma she was referred to hematology team and she was started on Bortezomib based chemotherapy.Conclusion: Pulmonary amyloidosis is an extremely rare entity and is commonly AL type.
Год издания:2021
Издательство:Wiley
Источник:Respirology
Ключевые слова:Pulmonary Hypertension Research and Treatments