Pericardial effusion as a crucial presentation of Erdheim-Chester disease in a hemodialysis patient: an overlooked diagnosis

Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis whose hallmark is tissue infiltration by CD68-positive, CD1a-negative and usually S-100 protein-positive foamy non-Langerhans histiocytes and mononuclear cells. Here, we report a hemodialysis (HD) patient who presented with fe...

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Veröffentlicht in:Clinical nephrology 2012-07, Vol.78 (1), p.81-84
Hauptverfasser: CHEN, Meng-Tsai, WANG, Shu-Ming, LIN, Shih-Yi, TING, I-Wen, LIU, Jiung-Hsiun, KUO, Huey-Liang, HUANG, Chiu-Ching
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container_end_page 84
container_issue 1
container_start_page 81
container_title Clinical nephrology
container_volume 78
creator CHEN, Meng-Tsai
WANG, Shu-Ming
LIN, Shih-Yi
TING, I-Wen
LIU, Jiung-Hsiun
KUO, Huey-Liang
HUANG, Chiu-Ching
description Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis whose hallmark is tissue infiltration by CD68-positive, CD1a-negative and usually S-100 protein-positive foamy non-Langerhans histiocytes and mononuclear cells. Here, we report a hemodialysis (HD) patient who presented with fever and pericardial effusion. We performed pericardiocentesis with pericardial biopsy and the histological findings indicated ECD. We administered intravenous methylprednisolone pulse therapy (250 mg/d) followed by oral prednisolone (50 mg/d). The patient's fever gradually subsided and there was no recurrence of pericardial effusion. This is the first report of an HD patient with ECD. We suggest that ECD be considered in the differential diagnosis of new HD patients who present with pericardial effusion, especially when this did not improve following increased dose of HD.
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Vascular system</topic><topic>Diabetic Nephropathies - complications</topic><topic>Diabetic Nephropathies - therapy</topic><topic>Diseases of the pericardium</topic><topic>Echocardiography</topic><topic>Erdheim-Chester Disease - complications</topic><topic>Erdheim-Chester Disease - diagnosis</topic><topic>Erdheim-Chester Disease - drug therapy</topic><topic>Fever - etiology</topic><topic>Heart</topic><topic>Humans</topic><topic>Injections, Intravenous</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Methylprednisolone - administration &amp; dosage</topic><topic>Middle Aged</topic><topic>Nephrology. 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subjects Administration, Oral
Biological and medical sciences
Biopsy
Cardiology. Vascular system
Diabetic Nephropathies - complications
Diabetic Nephropathies - therapy
Diseases of the pericardium
Echocardiography
Erdheim-Chester Disease - complications
Erdheim-Chester Disease - diagnosis
Erdheim-Chester Disease - drug therapy
Fever - etiology
Heart
Humans
Injections, Intravenous
Male
Medical sciences
Methylprednisolone - administration & dosage
Middle Aged
Nephrology. Urinary tract diseases
Pericardial Effusion - diagnosis
Pericardial Effusion - etiology
Pericardial Effusion - therapy
Pericardiocentesis
Prednisolone - administration & dosage
Pulse Therapy, Drug
Renal Dialysis
Steroids - administration & dosage
Treatment Outcome
Whole Body Imaging
title Pericardial effusion as a crucial presentation of Erdheim-Chester disease in a hemodialysis patient: an overlooked diagnosis
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