scholarly journals A very rare case of FLT3-D835 positive blastic plasmacytoid dendritic cell neoplasm

2020 ◽  
Vol 7 (4) ◽  
pp. 57-62
Author(s):  
Vlad Andrei Cianga ◽  
Cătălin Doru Dănăilă ◽  
Ion Antohe
Keyword(s):  
Dendritic Cell ◽  
Rare Case ◽  
Elderly Population ◽  
Hematological Malignancies ◽  
Plasmacytoid Dendritic Cell ◽  
The Elderly ◽  
Skin Lesions ◽  
Response To Treatment ◽  
Cell Neoplasm ◽  
Immuno Histochemistry

Blastic plasmacytoid dendritic cell neoplasms (BPDCNs) are extremely rare and aggressive hematological malignancies that derive from precursors of plasmacytoid dendritic cells (pDC) and frequently involve skin lesions and bone marrow infiltration. They mostly affect the elderly population and the prognosis is poor with the therapeutic choices currently available. Diagnosis is made with the help of tools such as immuno-histochemistry and flow cytometry. Here, we present a particular case of BPDCN with a positive FLT3-D835 mutation and we discuss the possible impact this may have on the evolution of the disease and response to treatment.

A rare case of blastic plasmacytoid dendritic cell neoplasm with deletion 7q.31, in the setting of heavy pre-treatment with alkylating chemotherapy

2016 ◽  
Vol 23 (7) ◽  
pp. 552-556 ◽  
Author(s):  
Varinder Kaur ◽  
Arjun Swami ◽  
Atrash Shebli ◽  
Sara Shalin ◽  
Muthu Veeraputhiran ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Dendritic Cell ◽  
Rare Case ◽  
De Novo ◽  
Plasmacytoid Dendritic Cell ◽  
Initial Response ◽  
Skin Lesions ◽  
Lymph Node Involvement ◽  
Lymphoid Leukemia ◽  
Cell Neoplasm

Blastic plasmacytoid dendritic cell neoplasm is rare myeloid malignancy clinically characterized by non-pruritic, violaceous and papulo-nodular skin lesions, together with bone marrow and lymph node involvement. Histologically, there is infiltration of dermis by neoplastic mono-nuclear CD4, CD56, CD123 co-expressing cells with epidermal sparing. Most commonly blastic plasmacytoid dendritic cell neoplasm presents as a de-novo condition, and treatment-related blastic plasmacytoid dendritic cell neoplasm is a rare phenomenon. Due to rarity of the disease, there is no established standard of care treatment. Both acute myeloid leukemia and acute lymphoid leukemia type induction regimens have been used for treatment of blastic plasmacytoid dendritic cell neoplasm, with initial response rate of 50%–80%. We present a rare case of therapy-associated blastic plasmacytoid dendritic cell neoplasm in a patient with remote history alkylating agent systemic therapy. A lag period of five to seven years and presence of deletion 7q.31 seen in bone marrow biopsy specimen in our patient are consistent with a likely therapy-associated etiology of his blastic plasmacytoid dendritic cell neoplasm.

scholarly journals Blastic plasmacytoid dendritic cell neoplasm: a rare case of gingival lesion with leukaemic presentation

2018 ◽  
pp. bcr-2018-224623 ◽  
Author(s):  
Massimo Viviano ◽  
Serena Cocca ◽  
Clelia Miracco ◽  
Stefano Parrini
Keyword(s):  
Dendritic Cell ◽  
Rare Case ◽  
Plasmacytoid Dendritic Cell ◽  
Cell Neoplasm

Skin lesions serve as clues to relapse of pediatric blastic plasmacytoid dendritic cell neoplasm

2018 ◽  
Vol 35 (2) ◽  
pp. e132-e135 ◽  
Author(s):  
Sean Dreyer ◽  
Suzanne Mednik ◽  
Allison Truong ◽  
Scott Worswick ◽  
Philip Scumpia ◽  
...  
Keyword(s):  
Dendritic Cell ◽  
Plasmacytoid Dendritic Cell ◽  
Skin Lesions ◽  
Cell Neoplasm

scholarly journals A Case of Plasmacytoid Dendritic Cell Leukemia

2013 ◽  
Vol 59 (2) ◽  
pp. 111-114
Author(s):  
Judit Beáta Köpeczi ◽  
I Benedek ◽  
Erzsébet Benedek ◽  
Enikő Kakucs ◽  
Aliz Tunyogi ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Dendritic Cell ◽  
Plasmacytoid Dendritic Cell ◽  
Initial Response ◽  
Skin Lesions ◽  
High Rate ◽  
Response To Treatment ◽  
Cell Leukemia ◽  
Cutaneous Lesions ◽  
Hla Dr

AbstractIntroduction: Plasmacytoid dendritic cell leukemia is a rare subtype of acute leukemia, which has recently been established as a distinct pathologic entity that typically follows a highly aggressive clinical course in adults. The aim of this report is to present a case of plasmacytoid dendritic cell leukemia due to its rarity and difficulty to recognize and diagnose it.Case report: We present a case of a 67 year-old man who presented multiple subcutaneous lesions on his face, neck, chest and upper extremities with reddish-brown, brown colour. In the bone marrow aspirate 83% of the blast cells were found. Immunophenotypically the blasts were positive for CD4, CD56, CD123 (high intensity), CD36, CD22, CD10 (10.42%), CD33, HLA-DR, CD7 (9.24%), CD38 (34.8%) and negative for CD13, CD64, CD14, CD16, CD15, CD11b, CD11c, CD3, CD5, CD2, CD8, CD19, CD20, CD34. The skin biopsy showed lymphohistiocytoid infiltration in the dermis. The patient was diagnosed with acute plasmacytoid dendritic cell leukemia and received polychemotherapy with rapid response of skin lesions and blastic infiltration of the bone marrow. After 3 courses of polychemotherapy the cutaneous lesions reappeared and multiplied. The blast infiltration in the bone marrow increased to 70%. A more aggressive polychemotherapy regimen was administered, but the patient presented serious complications (febrile neutropenia) and died in septic shock 8 months after the initiation of treatment.Conclusions: Immunophenotyping of blasts cells is indispensable in the diagnosis of plasmacytoid dendritic cell leukemia. The CD4+, CD56+, lin-, CD123 ++high, CD11c-, CD36+, HLA-DR+, CD34-, CD45+ low profile is highly suggestive for pDCL. The outcome of plasmacytoid dendritic cell leukemia is poor. Despite the high rate of initial response to treatment, early relapses occur and the patients die of disease progression.

scholarly journals Ileocecal Mucosal Involvement of Blastic Plasmacytoid Dendritic Cell Neoplasm without Skin Lesions

2018 ◽  
Vol 93 (5) ◽  
pp. 487-491
Author(s):  
Hyun Ju Choi ◽  
Jongha Park ◽  
Jin Lee ◽  
Mi Young Kim ◽  
Heui Jeong Jeong ◽  
...  
Keyword(s):  
Dendritic Cell ◽  
Plasmacytoid Dendritic Cell ◽  
Skin Lesions ◽  
Mucosal Involvement ◽  
Cell Neoplasm

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.

scholarly journals Treatment of blastic plasmacytoid dendritic cell neoplasm

Hematology ◽  
2016 ◽  
Vol 2016 (1) ◽  
pp. 16-23 ◽  
Author(s):  
Jill M. Sullivan ◽  
David A. Rizzieri
Keyword(s):  
Acute Myeloid Leukemia ◽  
Dendritic Cell ◽  
Myeloid Leukemia ◽  
Plasmacytoid Dendritic Cell ◽  
Allogeneic Transplantation ◽  
Skin Lesions ◽  
Lymphoid Leukemia ◽  
Variable Expression ◽  
Cell Neoplasm ◽  
Acute Myeloid

Abstract Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare myeloid malignancy with no defined standard of care. BPDCN presents most commonly with skin lesions with or without extramedullary organ involvement before leukemic dissemination. As a result of its clinical ambiguity, differentiating BPDCN from benign skin lesions or those of acute myeloid leukemia with leukemia cutis is challenging. BPDCN is most easily defined by the phenotype CD4+CD56+CD123+lineage–MPO–, although many patients will present with variable expression of CD4, CD56, or alternate plasmacytoid markers, which compounds the difficulty in differentiating BPDCN from other myeloid or lymphoid malignancies. Chromosomal aberrations are frequent, and the mutational landscape of BPDCN is being rapidly characterized although no obvious molecular target for chemoimmunotherapy has been identified. Chemotherapy regimens developed for acute myeloid leukemia, acute lymphoid leukemia, and myelodysplastic syndrome have all been used to treat BPDCN. Relapse is frequent, and overall survival is quite poor. Allogeneic transplantation offers a chance at prolonged remission and possible cure for those who are eligible; unfortunately, relapse remains high ranging from 30% to 40%. Novel therapies such as SL-401, a diphtheria toxin conjugated to interleukin-3 (IL-3) is commonly overexpressed in BPDCN and other aggressive myeloid malignancies and has shown considerable promise in ongoing clinical trials. Future work with SL-401 will define its place in treating relapsed or refractory disease as well as its role as a first-line therapy or bridge to transplantation.

scholarly journals Skin lesions in a patient with blastic plasmacytoid dendritic cell neoplasm

2013 ◽  
Vol 164 (6) ◽  
pp. 758-758 ◽  
Author(s):  
Ivan Dlouhy ◽  
Rodrigo Santacruz ◽  
Oscar Peña
Keyword(s):  
Dendritic Cell ◽  
Plasmacytoid Dendritic Cell ◽  
Skin Lesions ◽  
Cell Neoplasm
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