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

Abstract
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.
AuthorsMeng-Tsai Chen, Shu-Ming Wang, Shih-Yi Lin, I-Wen Ting, Jiung-Hsiun Liu, Huey-Liang Kuo, Chiu-Ching Huang
JournalClinical nephrology (Clin Nephrol) Vol. 78 Issue 1 Pg. 81-4 (Jul 2012) ISSN: 0301-0430 [Print] Germany
PMID22732342 (Publication Type: Case Reports, Journal Article)
Chemical References
  • Steroids
  • Prednisolone
  • Methylprednisolone
Topics
  • Administration, Oral
  • Biopsy
  • Diabetic Nephropathies (complications, therapy)
  • Echocardiography
  • Erdheim-Chester Disease (complications, diagnosis, drug therapy)
  • Fever (etiology)
  • Humans
  • Injections, Intravenous
  • Male
  • Methylprednisolone (administration & dosage)
  • Middle Aged
  • Pericardial Effusion (diagnosis, etiology, therapy)
  • Pericardiocentesis
  • Prednisolone (administration & dosage)
  • Pulse Therapy, Drug
  • Renal Dialysis
  • Steroids (administration & dosage)
  • Treatment Outcome
  • Whole Body Imaging

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