Among patients with Erdheim-Chester disease (ECD), it is important to evaluate adults with histiocytosis for a concomitant myeloid neoplasm, researchers concluded after conducting a multi-institutional cohort analysis involving 189 individuals.
Investigators looked at patients who had ECD and ECD overlapping with Langerhans cell histiocytosis. Among the results:
- 10% of patients with ECD had an overlapping myeloid neoplasm, typically a myeloproliferative neoplasm (MPN), myelodysplastic syndrome (MDS), or mixed MDS/MPN overlap syndrome.
- Molecular analysis often spotted hallmark driver mutations of myeloid neoplasms coexisting with those characteristic of histiocytosis.
- Patients with histiocytosis with a concomitant myeloid malignancy tended to be substantially older.
- They also were more likely to present with mixed histiocytosis than those without a myeloid malignancy.
- Discrete kinase mutations in the histiocytosis and myeloid neoplasm led to discordant and adverse responses to kinase-directed targeted therapies in selected cases.
Papo M, Diamond E, Cohen-Aubart F, et al. High prevalence of myeloid neoplasms in adults with non-Langerhans cell histiocytosis. Blood. 2017;130(8):1007-1013. doi:10.1182/blood-2017-01-761718.
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