Clearance of bone marrow mast cells after hypomethylating agent and venetoclax for systemic mastocytosis associated with myeloid neoplasia

Eur J Haematol. 2023 Feb;110(2):213-216. doi: 10.1111/ejh.13894. Epub 2022 Nov 15.

Abstract

Systemic mastocytosis (SM) results from clonal proliferation of neoplastic mast cells that infiltrate bone marrow and other organs. A major subset of patients with SM has a clonally related myeloid neoplasm and the SM itself (SM-AMN). We evaluated the efficacy of hypomethylating agent and venetoclax (HMA-VEN) to target both the myeloid neoplasm and mast cell infiltrate in a patient with SM associated with acute myeloid leukemia arising from myelodysplastic syndrome and illustrate complete elimination of bone marrow mast cells and complete remission of MDS/AML. This case illustrates the potent activity of HMA-VEN both against the AMN as well as the associated SM.

Keywords: hypomethylating agent; mastocytosis; venetoclax.

Publication types

  • Case Reports

MeSH terms

  • Bone Marrow
  • Humans
  • Leukemia, Myeloid, Acute* / complications
  • Leukemia, Myeloid, Acute* / diagnosis
  • Leukemia, Myeloid, Acute* / drug therapy
  • Mast Cells
  • Mastocytosis, Systemic* / complications
  • Mastocytosis, Systemic* / diagnosis
  • Mastocytosis, Systemic* / drug therapy
  • Myelodysplastic Syndromes* / complications
  • Myelodysplastic Syndromes* / diagnosis
  • Myelodysplastic Syndromes* / drug therapy
  • Myeloproliferative Disorders* / complications
  • Myeloproliferative Disorders* / diagnosis
  • Myeloproliferative Disorders* / drug therapy

Substances

  • venetoclax