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Erdheim-Chester disease associated with renal involvement and thrombocythemia

  • Case Reports
  • Musculoskeletal Radiology
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Abstract

Erdheim-Chester disease is a rare, distinctive lipoidosis characterised by deposition of cholesterol-laden foam cells in the bone marrow associated with a granulomatous reaction affecting the lungs, pericardium, heart and retroperitoneum. A case of Erdheim-Chester disease is presented, with typical skeletal involvement (i. e. diffuse sclerosis of the diaphyses and metaphyses of the long tubular bones) and atypical features including thrombocythemia and renal involvement which was documented on computed tomography. Other features of the reported case include gonadttrophin insufficiency and diabetes insipidus.

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Authors and Affiliations

  1. Department of Radiology, Hôpital La Pitié 83, Bd de L'Hôpital, 75651, Paris Cedex 13, France

    M. F. Bellin, E. Dion & J. Grellet

  2. Department Internal Medicine, Hôpital La Pitié 83, Bd de L'Hôpital, 75651, Paris Cedex 13, France

    P. Cacoub, B. Wechsler & P. Godeau

  3. Department of Pathology, Hôpital Henri Mondor, 51, Av du Mal De Lattre de Tassigny, 94010, Créteil Cedex, France

    J. Wechsler

  4. Department of Pathology, Hôpital Bicétre, 78 rue du Gl Leclerc, 94275, Le Kremlin Bicétre Cedex, France

    J. Quillard

Authors
  1. M. F. Bellin
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  2. P. Cacoub
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  3. J. Wechsler
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  4. J. Quillard
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  5. E. Dion
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  6. B. Wechsler
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  7. P. Godeau
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  8. J. Grellet
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Correspondence to: M. F. Bellin

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Bellin, M.F., Cacoub, P., Wechsler, J. et al. Erdheim-Chester disease associated with renal involvement and thrombocythemia. Eur. Radiol. 3, 266–269 (1993). https://doi.org/10.1007/BF00425909

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  • DOI: https://doi.org/10.1007/BF00425909

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