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ZBTB46

Friday 23 November 2018

ZBTB46 is a zinc finger and BTB domain-containing transcription factor, which is expressed by dendritic cells and committed dendritic cell precursors, but not by plasmacytoid dendritic cells, monocytes, macrophages, or other immune cell populations.

Pathology

- Expression of the transcription factor ZBTB46 distinguishes human histiocytic disorders of classical dendritic cell origin. (29743654)

  • In a study, all examined cases of Langerhans cell histiocytosis and histiocytic sarcoma expressed ZBTB46.
  • All cases of blastic plasmacytoid dendritic cell neoplasm, chronic myelomonocytic leukemia, juvenile xanthogranuloma, Rosai-Dorfman disease, and Erdheim-Chester disease failed to demonstrate expression of ZBTB46.
  • ZBTB46 expression clarifies the identity of diagnostically challenging neoplasms, such as cases of indeterminate cell histiocytosis, classifying a fraction of these entities as dendritic cell malignancies.
  • These findings clarify the lineage origins of human histiocytic disorders and distinguish dendritic cell disorders from all other myeloid neoplasms.

Open references

- Expression of the transcription factor ZBTB46 distinguishes human histiocytic disorders of classical dendritic cell origin. Satpathy AT, Brown RA, Gomulia E, BriseƱo CG, Mumbach MR, Pan Z, Murphy KM, Natkunam Y, Chang HY, Kim J. Mod Pathol. 2018 Sep;31(9):1479-1486. doi : 10.1038/s41379-018-0052-4 PMID: 29743654 Free