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

2016 ◽  
Vol 91 (3) ◽  
pp. 283-286 ◽  
Author(s):  
Uday Deotare ◽  
Karen W.L. Yee ◽  
Lisa W. Le ◽  
Anna Porwit ◽  
Anne Tierens ◽  
...  
Keyword(s):  
Flow Cytometry ◽  
Dendritic Cell ◽  
Plasmacytoid Dendritic Cell ◽  
Color Flow ◽  
Cell Neoplasm

scholarly journals A Case of Blastic Plasmacytoid Dendritic Cell Neoplasm Extensively Studied by Flow Cytometry and Immunohistochemistry

2017 ◽  
Vol 2017 ◽  
pp. 1-6
Author(s):  
Martina Pennisi ◽  
Clara Cesana ◽  
Micol Giulia Cittone ◽  
Laura Bandiera ◽  
Barbara Scarpati ◽  
...  
Keyword(s):  
Flow Cytometry ◽  
Dendritic Cell ◽  
Hematologic Malignancy ◽  
Lymphoblastic Leukemia ◽  
Plasmacytoid Dendritic Cell ◽  
Bone Marrow Involvement ◽  
Hematopoietic Stem ◽  
Cell Neoplasm ◽  
Specific Antigens ◽  
Aggressive Clinical Course

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy with aggressive clinical course and poor prognosis. Diagnosis is based on detection of CD4+CD56+,CD123high, TCL-1+, and blood dendritic cell antigen-2/CD303+blasts, together with the absence of lineage specific antigens on tumour cells. In this report we present a case of BPDCN presenting with extramedullary and bone marrow involvement, extensively studied by flow cytometry and immunohistochemistry, who achieved complete remission after acute lymphoblastic leukemia like chemotherapy and allogeneic hematopoietic stem cell transplantation.

The diagnostics of blastic plasmocytoid dendritic cell neoplasm: report of five cases

2021 ◽  
Vol 20 (3) ◽  
pp. 60-67
Author(s):  
I. A. Demina ◽  
S. A. Kashpor ◽  
O. I. Illarionova ◽  
M. E. Dubrovina ◽  
A. A. Dudorova ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Flow Cytometry ◽  
Dendritic Cell ◽  
Plasmacytoid Dendritic Cell ◽  
Skin Lesions ◽  
Flow Cytometry Data ◽  
Hematological Disorders ◽  
Pediatric Hematology ◽  
National Medical ◽  
Cell Neoplasm

The diagnosis of rare hematological disorders requires a comprehensive clinical and laboratory investigation with careful interpretation of all test results. Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is one of such rare entities. We have performed a retrospective analysis of the results of immunophenotyping, cytomorphology and cytogenetics of bone marrow tumor cells from 5 patients with BPDCN aged from 8 to 51 years. The study was approved by the Independent Ethics Committee of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. No specific characteristics of blasts were found. No correlation with the treatment and outcomes was noted as well: 3 patients died of progression or relapse (2 and 1, respectively). Bone marrow immunophenotyping is probably the most valuable laboratory test which allows physicians to establish the proper diagnosis in the absence of skin lesions. Flow cytometry immunophenotyping is the only technique used to determine the antigen profile that enables us to distinguish normal plasmacytoid dendritic cells from tumor ones by the presence (or absence) of the expression of CD2, CD7, CD38, CD56, CD303 etc. In the present paper, we provide a detailed description of five cases of BPDCN and main methods for flow cytometry data analysis. The parents of the patients agreed to use the information, including photos of children, in scientific research and publications.

Diagnosis of Blastic Plasmacytoid Dendritic Cell Neoplasm By Using 10-Colour Flow Cytometry and Achievement of Remission By Hypercvad Therapy

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 2365-2365 ◽  
Author(s):  
Uday Deotare ◽  
Elizabeth Hyjek ◽  
Anna Porwit ◽  
Rumina Musani ◽  
David Barth ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Flow Cytometry ◽  
Overall Survival ◽  
Dendritic Cell ◽  
Research Funding ◽  
Plasmacytoid Dendritic Cell ◽  
Leukemic Cells ◽  
Front Line ◽  
Hla Dr ◽  
Cell Neoplasm

Abstract Background: Although classified by WHO 2008 as belonging to the category “Acute myeloid leukemia and related precursor neoplasms”, Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) presents as an acute leukemia (AL) only in a minority of cases. There are only few studies describing the comprehensive immunophenotypic pattern of BPDCN in the bone marrow. Furthermore, given the rarity of this hematologic malignancy optimal frontline therapy is unclear. Patients and Methods: This retrospective analysis evaluates the diagnostic flow cytometry pattern and outcome of 9 patients who were diagnosed with BPDCN at the Princess Margaret Cancer Centre between December 2008 and June 2014. A four tube 10-color flow cytometry (FCM) panel has been used to correctly make the diagnosis of BPDCN in 6 patients, whereas a 5-colour panel was used in the remaining patients in conjunction with immunohistochemistry. The following markers were included in the10-color panel: Tube 1: CD65 FITC, CD13 PE, CD14 ECD, CD33 PC5.5, CD34 PC7, CD117 APC, CD7 A700, CD11b A750, CD16 PB, and CD45 KO; Tube 2: CD36 FITC, CD64 PE, CD56 ECD, CD33 PC5.5, CD34 PC7, CD123 APC, CD19 A700, CD38 A750, HLA-DR PB, and CD45 KO; Tube 3: CD71 FITC, CD11c PE, CD4 ECD, CD33 PC5.5, CD34 PC7, CD2 APC, CD10 A700, CD235a A750, CD15 PB, and CD45 KO; Tube 4:nuclear (n) TdT FITC, cytoplasmic (cyt.) MPO PE, CD14 ECD, CD33 PC5.5, CD34 PC7, cyt.CD79a APC, cyt.CD22 A700, CD19 A750, cyt.CD3 PB, and CD45 KO. Results: Median age was 66 years (range, 25 to 91 years); 3 patients were over the age of 70 years. Fifty-six percent were males. All presented with skin lesions and 78% presented each with lymphadenopathy and bone marrow involvement. Cytogenetics were poor-risk in 2 patients, intermediate-risk in 3 and unknown or inconclusive in 4. By 10-color FCM, leukemic cells were in the blast gate (CD45dim/low SSC) and were positive for CD4(bright), CD33(dim), CD56(heterogenous), CD123(bright), CD36, CD38, HLA-DR, CD71, but negative for CD10, CD11b, CD13, CD14, CD15, CD16, CD19, CD34, CD64, CD65, CD235a. Other markers, such as cyt.MPO, cyt.CD3, cyt.CD22 and nTdT were negative, while dim cyt.CD79a was seen in 3 cases. CD7 expression was found in 5 cases, whereas CD2 and CD117 were found in single cases only. BM involvement by BPDCN leukemic cells ranged from 27% to 92% of the marrow cellularity. Skin involvement showed dense infiltrate of cells with blastoid morphology and characteristic grenz zone. Seven patients received front-line induction therapy with HyperCVAD with an overall response rate of 86% (4 complete remissions (CR), 2 unconfirmed CRs). One patient died of multi-organ failure during induction. Three of 6 responders underwent planned allogeneic hematopoietic cell transplantation (HCT); 1 patient has since died of acute graft versus host disease (GVHD), whereas 2 are alive in remission with chronic GVHD, 12 and 14 months post transplant with complete donor chimerism. One transplant ineligible patient relapsed 22 months after achievement of CR1. Median follow-up of all patients was 12 months with a overall survival at 1 year of 59.3% for the entire group. Patients who underwent allogeneic HCT had overall survival at 1 year of 66.7% and for the chemotherapy group was 27.8% at 1 year.(p=0.34). Conclusion: An accurate diagnosis of BPDCN can be made by 10-colour FCM using a 4-tube acute leukemia panel. BPDCN demonstrates a characteristic pattern of antigen expression . Although front-line induction chemotherapy with HyperCVAD can yield high CR rates, allogeneic HCT should be performed in first CR for transplant eligible patients, as this appears to be required for long term durable remissions. For transplant ineligible or relapsed BPDCN patients, there is an unmet need for novel therapeutic agents. Disclosures Porwit: Beckman-Coulter: Speakers Bureau. Gupta:Novartis: Consultancy, Honoraria, Research Funding; Incyte Corporation: Consultancy, Research Funding.

scholarly journals Flow cytometry to identify bone-marrow relapse in blastic plasmacytoid dendritic cell neoplasm: a case report

2017 ◽  
Vol 39 (3) ◽  
pp. 274-277
Author(s):  
Mariela Granero Farias ◽  
Fabiane Spagnol Pedrazzani ◽  
Luis Carlos Zanandrea Contin ◽  
Ana Paula Alegretti ◽  
Lisandra Della Costa Rigoni ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Flow Cytometry ◽  
Case Report ◽  
Dendritic Cell ◽  
Plasmacytoid Dendritic Cell ◽  
Cell Neoplasm

scholarly journals Relapse of tagraxofusp treated blastic plasmacytoid dendritic cell neoplasm with loss of CD123 expression

2021 ◽  
Author(s):  
Rohit Gulati ◽  
Asma Abu-Salah ◽  
Tareq Salous ◽  
Mehdi Nassiri
Keyword(s):  
Bone Marrow ◽  
Flow Cytometry ◽  
Dendritic Cell ◽  
Plasmacytoid Dendritic Cell ◽  
Primary Treatment ◽  
Resistance Mechanisms ◽  
Elderly Male ◽  
Bone Marrow Specimen ◽  
Cell Neoplasm ◽  
Targeted Immunotherapy

AbstractTagraxofusp, a CD123-based-targeted immunotherapy, was recently approved to treat blastic plasmacytoid dendritic cell neoplasm (BPDCN) with excellent response. Also, a subset of BPDCN shows resistance to tagraxofusp. These resistant cases continue to express CD123, which forms the basis of the continued utility of tagraxofusp in newer combination chemotherapies to overcome resistance in BPDCN. Herein, we report a case of an elderly male with BPDCN that achieved complete remission on initial primary treatment with tagraxofusp. However, BPDCN relapsed after 1.5 years while on treatment, with loss of CD123 expression. At relapse, the neoplasm was comprehensively immunophenotyped by flow cytometry (performed on both peripheral blood and bone marrow specimen) and by immunohistochemical evaluation of the bone marrow clot section. The neoplasm at relapse was diagnostic of BPDCN with a lack of CD123 expression. This case highlights a potential limitation of current and upcoming tagraxofusp-based multidrug therapies, at least in a subset of refractory BPDCN. We believe our report will serve as a sentinel to incite future investigations involving alternate resistance mechanisms in BDPCN.

scholarly journals Blastic plasmacytoid dendritic cell neoplasm

2013 ◽  
Vol 88 (6 suppl 1) ◽  
pp. 158-161 ◽  
Author(s):  
André Lencastre ◽  
Joana Cabete ◽  
Alexandre João ◽  
Pedro Farinha ◽  
Gilda Ferreira ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Flow Cytometry ◽  
Dendritic Cell ◽  
Plasmacytoid Dendritic Cell ◽  
Flow Cytometry Analysis ◽  
Bone Marrow Biopsies ◽  
Cervical Mass ◽  
Cell Neoplasm ◽  
History Of ◽  
Thoracic And Abdominal

Blastic plasmacytoid dendritic cell neoplasm is a rare and aggressive hematodermic neoplasia with frequent cutaneous involvement and leukemic dissemination. We report the case of a 76-year-old man with a 2 month history of violaceous nodules and a tumor with stony consistency, located on the head, and mandibular, cervical and supraclavicular lymphadenopathies. Multiple thoracic and abdominal adenopathies were identified on computerized tomography. Flow cytometry analysis of the skin, lymph node and bone marrow biopsies demonstrated the presence of plasmocytoid dendritic cell neoplastic precursor cells (CD4+, CD45+, CD56+ and CD123+ phenotype). After initial clinical and laboratorial complete remission with chemotherapy, the patient died due to relapse of the disease associated with the appearance of a cervical mass with medullary compromise.

Tagraxofusp and anti-CD123 in blastic plasmacytoid dendritic cell neoplasm: a new hope

2020 ◽  
Vol 111 (5) ◽  
Author(s):  
Delia Cangini ◽  
Paolo Silimbani ◽  
Alessandro Cafaro ◽  
Maria B. Giannini ◽  
Carla Masini ◽  
...  
Keyword(s):  
Dendritic Cell ◽  
Plasmacytoid Dendritic Cell ◽  
Cell Neoplasm
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