16 November 2010
Anasarca and chylous effusions: An unusual presentation of systemic amyloidosis
Maureen R. HortonAm J Case Rep 2010; 11:191-194 :: ID: 881255
Abstract
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
In Press
Case report
Am J Case Rep In Press; DOI: 10.12659/AJCR.946911
Case report
Am J Case Rep In Press; DOI: 10.12659/AJCR.947730
Case report
Am J Case Rep In Press; DOI: 10.12659/AJCR.946625
Case report
Am J Case Rep In Press; DOI: 10.12659/AJCR.946795
Most Viewed Current Articles
21 Jun 2024 : Case report
98,661
DOI :10.12659/AJCR.944371
Am J Case Rep 2024; 25:e944371
07 Mar 2024 : Case report
53,011
DOI :10.12659/AJCR.943133
Am J Case Rep 2024; 25:e943133
20 Nov 2023 : Case report
33,935
DOI :10.12659/AJCR.941424
Am J Case Rep 2023; 24:e941424
07 Jul 2023 : Case report
23,965
DOI :10.12659/AJCR.940200
Am J Case Rep 2023; 24:e940200