Blastic plasmacytoid dendritic cell neoplasm
Updates to Article Attributes
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare haematological malignancy. It was previously termed as blastic natural-killer lymphoma or agranular CD4+ natural killer cell leukemia.
Epidemiology
It only represent a wel small propotion (around 0.44%) of all haematological malignancies 4. 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 leukaemic presenation 5.
Pathology
Histologically, the tumour cells may be either blastoid or pleomorphic. The 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 4.
Other involvement
Lymphadenopathy and hepatosplenomegaly may be present.
Nuclear medicine
FDG-PET
Skin lesions and lymph node metastases may appear hypermetabolic on F18-FDG PET imaging. Positive bone marrow involvement, however, may not be always evident on F18-FDG PET 4.
CT
Pulmonary involvement
Has been described as interstitial opacities with a ground glass and reticular opacities 4.
Other involvement
Lympadenopathy and hepatosplenomegaly may be present.
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 4.
See also
-</ul><h4>Radiographic features</h4><h5>FDG-PET</h5><p>Skin lesions and lymph node metastases may appear hypermetabolic on F18-FDG PET imaging. Positive bone marrow involvement however may not be always evident on F18-FDG PET <sup>4</sup>.</p><h5>CT </h5><h6>Pulmonary involvement</h6><p>Has been described as interstitial opacities with a ground glass and reticular opacities<sup> 4</sup>.</p><h6>Other involvement</h6><p>Lympadenopathy and hepatosplenomegaly may be present.</p><h4>Treatment and prognosis</h4><p>Chemotherapy is the preferred treatment, along with allogeneic stem cell transplantation. Patients usually have poor outcome with a median survival reported around 12-14 months. Advanced stage and older age may imply poorer prognosis<sup> 4</sup>.</p><h4>See also</h4><ul><li><a href="/articles/2008-who-classification-of-tumours-of-haematopoietic-and-lymphoid-tissues">2008 WHO classification of tumours of haematopoietic and lymphoid tissues</a></li></ul>- +</ul><h4>Radiographic features</h4><h5>CT </h5><h6>Pulmonary involvement</h6><p>Has been described as interstitial opacities with a <a title="Ground glass" href="/articles/ground-glass-opacification-1">ground glass</a> and <a title="Reticular opacities" href="/articles/reticular-interstitial-pattern">reticular opacities</a><sup> 4</sup>.</p><h6>Other involvement</h6><p><a title="Lymphadenopathy" href="/articles/lymph-node-enlargement">Lymphadenopathy</a> and hepatosplenomegaly may be present.</p><h5>Nuclear medicine</h5><h6>FDG-PET</h6><p>Skin lesions and lymph node metastases may appear hypermetabolic on F18-FDG PET imaging. Positive bone marrow involvement, however, may not be always evident on F18-FDG PET <sup>4</sup>.</p><h4>Treatment and prognosis</h4><p>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<sup> 4</sup>.</p><h4>See also</h4><ul><li><a href="/articles/2008-who-classification-of-tumours-of-haematopoietic-and-lymphoid-tissues">2008 WHO classification of tumours of haematopoietic and lymphoid tissues</a></li></ul>
Systems changed:
- Oncology
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