Background: Amyloidosis is the result of pathologic deposition of insoluble fibrilar proteins in normal tissues. The resultant accumulation of proteins leads to a wide array of seeming unconnected organ dysfunctions and symptoms often delaying diagnosis.
Case Report: We present a case of primary systemic amyloidosis presenting as anasarca and chylous effusions in a 63 year old man with a past medical history of hypertension and obstructive sleep apnea. The patient noted the onset of anasarca, dyspnea on exertion in the setting of a 50-pound weight loss over 6 months. Through evaluation revealed anasarca, chylous pleural and peritoneal effusions, tachypnea and dyspnea on exertion. Laboratories were unremarkable except for an elevated ESR of 30 and BUN/Cr of 27/1.6 with a bland urinalysis. Chest imaging revealed bilateral pleural effusions with compressive atelectasis and lymphoscintigraphy revealed normal lymphatic flow with no thoracic duct leak. Due to concerns for constrictive pericarditis, the patient underwent a right heart catheterization that was not consistent with pericardial disease. However, endomyocardial biopsy revealed cardiac amyloidosis. Further evaluation revealed elevated free serum Kappa light chains with an elevated free Kappa/Lambda ratio.
Conclusions: We present an atypical presentation of a rare disease, primary AL amyloidosis. Although large pleural effusions are rare in amyloidosis, they are usually exudative and may be due to direct pleural infiltration or amyloid–induced heart, liver, renal or thyroid failure. Thus, large pleural effusions, especially chylous, without clear etiology should raise the suspicion for amyloidosis and lead to further investigations.

Keywords: chylous, anasarca, Amyloidosis