Systemic Mastocytosis Associated with "Smoldering" Multiple Myeloma.
Magda ZanelliStefano RicciMaurizio ZizzoFrancesca SanguedolceFederica De GiorgiAndrea PalicelliGiovanni MartinoStefano AscaniPublished in: Diagnostics (Basel, Switzerland) (2021)
A 79-year-old woman presented with a long history of peripheral eosinophilia. Previous right hemicolectomy for colonic polyposis was reported. Laboratory tests were notable for mild macrocitic anaemia and eosinophilia. β2 microglobulin and serum tryptase levels were elevated. Serum immunofixation revealed IgA/kappa monoclonal protein. Bence-Jones protein was positive. Bone marrow (BM) biopsy revealed the coexistence of two neoplastic components. Cohesive clusters of bland-looking, spindle-shaped mast cells, representing 20% of marrow cellularity, were close to aggregates of mature plasma cells occupying 40% of marrow cellularity. Molecular analysis on marrow aspirate demonstrated KIT D816V mutation, TET2 mutation, monoallelic deletion of TP53/17p13 and trisomy of ATM/11q23. A bone density study revealed mild osteoporosis. Full skeletal X-rays and magnetic resonance imaging (MRI) of spine and hips showed multiple, small rarefaction areas and an old L1-L2 fracture, both ascribed to osteoporosis. The association of systemic mastocytosis (SM) and multiple myeloma (MM) is very uncommon. The coexistence of SM with MM placed our patient in the SM with associated clonal haematological non-mast-cell lineage disease (SM-AHN) subtype. Midostaurin therapy was started.
Keyphrases
- multiple myeloma
- magnetic resonance imaging
- single cell
- bone mineral density
- bone marrow
- postmenopausal women
- contrast enhanced
- induced apoptosis
- protein protein
- computed tomography
- cell cycle arrest
- mesenchymal stem cells
- nuclear factor
- amino acid
- dna damage
- case report
- body composition
- stem cells
- dna repair
- cell death
- diffusion weighted imaging
- magnetic resonance
- oxidative stress
- bone loss
- soft tissue