Conflict of interest: Nothing to report.
Images in Hematology
Blastic plasmacytoid dendritic cell neoplasm†
Article first published online: 26 SEP 2011
Copyright © 2011 Wiley Periodicals, Inc.
American Journal of Hematology
Volume 87, Issue 7, page 710, July 2012
How to Cite
Munoz, J., Rana, J., Inamdar, K., Nathanson, D. and Janakiraman, N. (2012), Blastic plasmacytoid dendritic cell neoplasm . Am. J. Hematol., 87: 710. doi: 10.1002/ajh.22173
- Issue published online: 14 JUN 2012
- Article first published online: 26 SEP 2011
- Accepted manuscript online: 26 AUG 2011 12:00AM EST
- Manuscript Accepted: 18 AUG 2011
- Manuscript Revised: 8 AUG 2011
- Manuscript Received: 23 JUN 2011
Sixty-six-year-old female presented with left forearm soft tissue swelling for 2 months. An ultrasound of her left forearm showed a 6.5 cm × 1 cm × 3.6 cm soft tissue mass, which was also confirmed by magnetic resonance imaging (Panel A and B). A routine screening mammogram revealed a new 4 mm mass in her left breast. Subsequent left breast needle biopsy and left forearm fine needle aspiration favored an undifferentiated malignant neoplasm of hematolymphoid origin (Panel D) with positive CD4 (Panel E) and positive CD56 (Panel F) markers compatible with blastic plasmacytoid dendritic cell neoplasm (BPDCN). Positron emission tomography (PET) scan showed increased uptake in the left forearm (Panel C), left breast, bilateral pleura, liver, spleen, portocaval lymph nodes, and omental caking. A bone marrow biopsy was negative for malignancy, and shortly thereafter the patient underwent induction therapy with cytarabine and idarubicin. Following progression of metastatic disease on PET scan, the patient received reinduction chemotherapy with mitoxantrone, etoposide, and cytarabine.
BPDCN is a rare malignancy formerly recognized as CD4+/CD56+ hematodermic neoplasm and is suggested to have derived from plasmacytoid dendritic cells. It typically presents in middle-aged or elderly patients with skin or soft tissue involvement and concurrent disseminating disease carrying poor prognosis. BPDCN expresses CD4, CD56, CD123 (interleukin-3 receptor alpha chain), and BDCA-2 (blood dendritic cell antigen 2), whereas expression of TCL1 is helpful when the tissue displays weak expression of aforementioned markers. Treatment involves multiagent chemotherapy followed by subsequent stem cell transplantation for best outcomes. Unfortunately, our patient showed dismal response to chemotherapy, and the originally intended stem cell transplantation could not be instituted due to poor performance status.
- 2Blastic plasmacytoid dendritic cell neoplasm: is transplantation the treatment of choice? Br J Dermatol. 2010 Jan; 162(1): 74–9. Epub 2009 Jun 22., , , et al.