16 November 2010
Am J Case Rep 2010; 11:191-194 :: ID: 881255
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
22 September 2022 : Case reportRare Case of Persistently Depressed T Lymphocyte Subsets After SARS-CoV-2 Infection
Am J Case Rep In Press; DOI: 10.12659/AJCR.937760
22 September 2022 : Case reportCentral Retinal Vein Occlusion After Discontinuation of Rivaroxaban Therapy in a Young Patient with COVID-1...
Am J Case Rep In Press; DOI: 10.12659/AJCR.937739
19 September 2022 : Case reportInhaled Nitric Oxide in Acute Severe Pulmonary Hypertension and Severe Acute Respiratory Distress Syndrome ...
Am J Case Rep In Press; DOI: 10.12659/AJCR.937147
27 Sep 2022 : Case reportCompound Heterozygote Mutation in the SMPD1 Gene Leading to Nieman-Pick Disease Type A
Am J Case Rep In Press; DOI: 10.12659/AJCR.937220
26 Sep 2022 : Case reportAn Infant Presenting with Interstitial Lung Disease Diagnosed Later as Hunter Syndrome: A Case Report
Am J Case Rep In Press; DOI: 10.12659/AJCR.937527
26 Sep 2022 : Case reportAcute Intermittent Porphyria: Complete Phenotype in a Patient with p.Arg173Trp Variant in Thailand
Am J Case Rep In Press; DOI: 10.12659/AJCR.937695
Most Viewed Current Articles
13 Jul 2022 : Case reportWhistling Scrotum: An Unusual Presentation of Pneumomediastinum in the Setting of an Open Scrotal Wound
Am J Case Rep 2022; 23:e936441
23 Feb 2022 : Case reportPenile Necrosis Associated with Local Intravenous Injection of Cocaine
Am J Case Rep 2022; 23:e935250
06 Dec 2021 : Case reportLipedema Can Be Treated Non-Surgically: A Report of 5 Cases
Am J Case Rep 2021; 22:e934406