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Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation : 10-Color flow cytometry diagnosis and HyperCVAD therapy

Deotare, Uday ; Yee, Karen W.L. ; Le, Lisa W. ; Porwit, Anna LU ; Tierens, Anne ; Musani, Rumina ; Barth, David ; Torlakovic, Emina ; Schimmer, Aaron and Schuh, Andre C. , et al. (2016) In American Journal of Hematology 91(3). p.283-286
Abstract

Few studies describe the comprehensive immunophenotypic pattern of blastic plasmacytoid dendritic cell neoplasm (BPDCN) in the bone marrow and its treatment. This retrospective analysis evaluates the diagnostic flow cytometry (FCM) pattern and outcome of nine patients diagnosed with BPDCN. A four-tube 10-color FCM panel used for diagnosis of acute leukemia (AL), showed cells in the blast gate (CD45dim/low SSC) and were positive for CD4(bright), CD33(dim), CD56(heterogenous), CD123(bright), CD36, CD38, HLA-DR, CD71. Seven patients received front-line induction therapy with HyperCVAD with an overall response rate of 86%. Five of six responders underwent planned allogeneic hematopoietic cell transplantation (allo-HCT). For a median follow... (More)

Few studies describe the comprehensive immunophenotypic pattern of blastic plasmacytoid dendritic cell neoplasm (BPDCN) in the bone marrow and its treatment. This retrospective analysis evaluates the diagnostic flow cytometry (FCM) pattern and outcome of nine patients diagnosed with BPDCN. A four-tube 10-color FCM panel used for diagnosis of acute leukemia (AL), showed cells in the blast gate (CD45dim/low SSC) and were positive for CD4(bright), CD33(dim), CD56(heterogenous), CD123(bright), CD36, CD38, HLA-DR, CD71. Seven patients received front-line induction therapy with HyperCVAD with an overall response rate of 86%. Five of six responders underwent planned allogeneic hematopoietic cell transplantation (allo-HCT). For a median follow up of 13.3 months, the 1-year disease free survival and overall survival were 56 and 67%, respectively. An accurate diagnosis of BPDCN can be made by 10-color FCM using a four-tube AL panel demonstrating a characteristic pattern of antigen expression. Front-line induction chemotherapy with HyperCVAD can yield high remission rates, but allo-HCT is required for long-term durable remissions.

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publishing date
type
Contribution to journal
publication status
published
subject
in
American Journal of Hematology
volume
91
issue
3
pages
4 pages
publisher
John Wiley & Sons Inc.
external identifiers
  • pmid:26619305
  • scopus:84959161871
ISSN
0361-8609
DOI
10.1002/ajh.24258
language
English
LU publication?
no
id
faa83da1-1223-4b47-8668-b7e4e468f63a
date added to LUP
2019-05-22 09:39:55
date last changed
2024-04-30 09:15:48
@article{faa83da1-1223-4b47-8668-b7e4e468f63a,
  abstract     = {{<p>Few studies describe the comprehensive immunophenotypic pattern of blastic plasmacytoid dendritic cell neoplasm (BPDCN) in the bone marrow and its treatment. This retrospective analysis evaluates the diagnostic flow cytometry (FCM) pattern and outcome of nine patients diagnosed with BPDCN. A four-tube 10-color FCM panel used for diagnosis of acute leukemia (AL), showed cells in the blast gate (CD45dim/low SSC) and were positive for CD4(bright), CD33(dim), CD56(heterogenous), CD123(bright), CD36, CD38, HLA-DR, CD71. Seven patients received front-line induction therapy with HyperCVAD with an overall response rate of 86%. Five of six responders underwent planned allogeneic hematopoietic cell transplantation (allo-HCT). For a median follow up of 13.3 months, the 1-year disease free survival and overall survival were 56 and 67%, respectively. An accurate diagnosis of BPDCN can be made by 10-color FCM using a four-tube AL panel demonstrating a characteristic pattern of antigen expression. Front-line induction chemotherapy with HyperCVAD can yield high remission rates, but allo-HCT is required for long-term durable remissions.</p>}},
  author       = {{Deotare, Uday and Yee, Karen W.L. and Le, Lisa W. and Porwit, Anna and Tierens, Anne and Musani, Rumina and Barth, David and Torlakovic, Emina and Schimmer, Aaron and Schuh, Andre C. and Seftel, Matthew and Minden, Mark D. and Gupta, Vikas and Hyjek, Elizabeth}},
  issn         = {{0361-8609}},
  language     = {{eng}},
  month        = {{03}},
  number       = {{3}},
  pages        = {{283--286}},
  publisher    = {{John Wiley & Sons Inc.}},
  series       = {{American Journal of Hematology}},
  title        = {{Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation : 10-Color flow cytometry diagnosis and HyperCVAD therapy}},
  url          = {{http://dx.doi.org/10.1002/ajh.24258}},
  doi          = {{10.1002/ajh.24258}},
  volume       = {{91}},
  year         = {{2016}},
}