Blastic plasmacytoid dendritic cell neoplasm
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematological malignancy. It was previously termed as blastic natural-killer lymphoma or agranular CD4+ natural killer cell leukemia.
Epidemiology
It only represent a very small proportion (~0.44%) of all hematological malignancies . There may be a slight male predilection.
Clinical presentation
It often presents in the skin. Clinical course can be aggressive in adults and apparently milder in children. Some patient may have a leukemic presentation .
Pathology
Histologically, the tumor cells may be either blastoid or pleomorphic. They express several markers of plasmacytoid dendritic cells which include:
- CD123
- TCL1
- BDCA2
- CD2AP
Radiographic features
CT
Pulmonary involvement
Has been described as interstitial opacities with a ground glass and reticular opacities.
Other involvement
Lymphadenopathy and hepatosplenomegaly may be present.
Nuclear medicine
FDG-PET
Skin lesions and lymph node metastases may appear hypermetabolic on F-FDG PET imaging. Positive bone marrow involvement, however, may not be always evident on F-FDG PET .
Treatment and prognosis
Chemotherapy is the preferred treatment, along with allogeneic stem cell transplantation. Patients usually have a poor outcome with a median survival reported around 12-14 months. Advanced stage and older age may imply poorer prognosis.
See also
