Clinical Edge

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Look for Myeloid Neoplasm in These Patients

Blood; ePub 2017 Jul 5; Papo, Diamond, et al

It is important to evaluate adults with Erdheim-Chester disease (ECD) for a possible concomitant myeloid neoplasm, according to a multi-institutional cohort study involving 189 individuals.

Investigators looked at the occurrence of myeloid neoplasms in participants with ECD or ECD overlapping with mixed histiocytosis (MH). Among the results:

  • 1 in every 10 patients had an overlapping myeloid neoplasm.
  • Most were in the form of myeloproliferative neoplasm, myelodysplastic syndrome, or a combination of both.
  • Molecular analysis detected hallmark driver mutations of myeloid neoplasms co-existing with those characteristic of histiocytosis.
  • Patients with histiocytosis and diagnosed with a related myeloid malignancy were significantly older at diagnosis than those without a myeloid malignancy.
  • They also were more apt to present with MH.
  • In some cases, distinct kinase mutations in the histiocytosis and myeloid neoplasm resulted in discordant and adverse responses to kinase-directed targeted therapies.


Papo M, Diamond E, Cohen-Aubart F, et al. High prevalence of myeloid neoplasms in adults with non-Langerhans cell histiocytosis. [Published online ahead of print July 5, 2017]. Blood. doi:10.1182/blood-2017-01-761718.