[A case of systemic primary amyloidosis with BHL and M-protein in pleural effusion]

A 76-year-old man was admitted to our hospital because of productive cough and dyspnea. Chest X-ray revealed cardiomegaly, bilateral pleural effusion, mediastinal lymphadenopathy, and marked bilateral hilar lymphadenopathy (BHL). His symptoms improved after therapy including diuretic for heart failure, but BHL was unchanged. M-protein in both serum and pleural effusion, and B-J protein in urine were detected. Protein immunoelectrophoresis demonstrated a heterogeneous increase in IgA with lambda light chain predominance. Bronchoscopy disclosed some prominent mucosal lesions of the bilateral bronchi, of which biopsies demonstrated amyloid deposition. Gastric mucosal biopsy also demonstrated amyloid deposition. Therefore this case was diagnosed as systemic primary amyloidosis due to AL-protein. In systemic amyloidosis, there are only a few reports of lymph node enlargement due to amyloid deposition, and it is particularly rare with BHL. From the presence of M-protein in pleural effusion, it is suggested that not only congestive heart failure due to cardiac amyloidosis but also amyloid deposition in the pleura would be related to the fluid production.