The clinical spectrum of Erdheim-Chester disease: an observational cohort study.

@article{EstradaVeras2017TheCS,
  title={The clinical spectrum of Erdheim-Chester disease: an observational cohort study.},
  author={Juvianee I Estrada-Veras and Kevin J O'Brien and Louisa C. Boyd and Rahul Dave and Benjamin H. Durham and Liqiang Xi and Ashkan A. Malayeri and Marcus Y. Chen and Pamela J. Gardner and Jhonell R Alvarado-Enriquez and Nikeith Shah and Omar Abdel-Wahab and Bernadette R Gochuico and Mark A. Raffeld and Elaine S. Jaffe and William A Gahl},
  journal={Blood advances},
  year={2017},
  volume={1 6},
  pages={
          357-366
        }
}
Erdheim-Chester Disease (ECD) is a rare, potentially fatal, multi-organ myeloid neoplasm occurring mainly in adults. The diagnosis is established by clinical, radiologic, and histologic findings; ECD tumors contain foamy macrophages that are CD68+, CD163+, CD1a-, and frequently S100-. The purpose of this report is to describe the clinical and molecular variability of ECD. Sixty consecutive ECD patients (45 males, 15 females) were prospectively evaluated at the NIH Clinical Center between 2011… CONTINUE READING

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