Histiocyt en dentrische cel neoplasma
WHO 2022: HISTIOCYTIC/DENDRITIC CELL NEOPLASMS
These neoplasms are positioned in the classification WHO 2022 after myeloid neoplasms in recognition of their derivation from common myeloid progenitors that give rise to cells of the monocytic/histiocytic/dendritic lineages.
Key changes in the current edition of the classification include:
inclusion of clonal plasmacytoid dendritic cell (pDC) diseases in this category;
moving follicular dendritic cell sarcoma and fibroblastic reticular cell tumor to a separate category; and
addition of Rosai-Dorfman disease (RDD) and ALK-positive histiocytosis as disease types.
BPCDN
Blastic plasmacytoid dendritic cell neoplasm.
ECD
Achtergrond:
ECD: klonale proliferatie van histiocyten, histiocyten hebben vaak schuimig cytoplasma.
Kliniek
In >95% is er ossale betrokkenheid van lange pijpbeenderen
In ws 50% cardiovasculaire betrokkenheid (coated aorta)
In 33% retroperitoneale fibrose (hairy kidney)
In 20-30 CNS betrokkenheid, diabetes insipidus agv hypofyse infiltratie, exolftalmus agv orbitale infiltratie (vaak bilateraal). Cutane manifestaties zijn vooral xantalasmata.
Moleculaire:
In >50% BRAFV600E
Zeldzamer: NRAS (4%)
Vaak activerende mutaties in MAPK pathway
Workup
CT whole body: aanwijzingen voor ossale betrokkenheid?
Opvragen beeldvorming, oorzaal diabetes insipidus OLVG
PM. Cardiaal: ECG. Lab: NTproBNP? MRI hart?
Beleid als ECD
Interferon?