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[Erdheim-Chester disease: an aggressive systemic disease treated with cladribine with favorable outcome (a case

Najat Lasri1, Fatimaezzahra Lahlimi1, Mohammed Ilias Tazi1

  • 1Service d'Hématologie Clinique et de Greffe de Moelle, Centre Hospitalier Universitaire Mohammed VI, Faculté de Médecine et de Pharmacie, Université Cadi Ayyad, Marrakech, Maroc.

The Pan African Medical Journal
|August 1, 2022
PubMed

Insights

Erdheim-Chester disease (ECD), a rare histiocytosis, presents varied symptoms. This case highlights successful treatment of aggressive systemic ECD with cladribine, offering hope for patients with this challenging condition.

Area of Science:

  • Oncology
  • Pathology
  • Neurology

Background:

  • Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis with poorly understood pathophysiology.
  • Clinical manifestations of ECD are diverse, and diagnosis relies on pathological examination.
  • The 'hair kidney' sign on CT scans is a characteristic diagnostic indicator.

Keywords:
Erdheim-Chester diseaseNon-langerhanian histiocytosiscase reportcladribinesystemic disease

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