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.
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Long-term ibrutinib data in older patients, Barr PM et al. Haematologica. 2018;103(9):1502-10
Prognostic Score System for Patients with PMF, J Clin Oncol; ePub 2017 Dec 9; Gugliemelli, et al
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