scholarly journals Immunohistochemistry Aids in the Diagnosis of Blastic Plasmacytoid Dendritic Cell Neoplasm in a Patient with Multiple Cutaneous Plaques and Nodules

2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S47-S47
Author(s):  
H Kaur ◽  
C Reyes-Barron ◽  
G Scott
Keyword(s):  
Bone Marrow ◽  
T Cell ◽  
Dendritic Cell ◽  
Hematologic Malignancy ◽  
Plasmacytoid Dendritic Cell ◽  
Care Physician ◽  
Cutaneous Lesions ◽  
T Cell Lymphomas ◽  
Cell Neoplasm ◽  
Dermal Infiltrate

Abstract Introduction/Objective Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) is a rare hematologic malignancy of plasmacytoid dendritic cell precursors with an estimated incidence of 0.04 cases per 100,000 in the US. Given the cutaneous tropism of BPDCN, it should be differentiated from other CD56+ hematopoietic neoplasms with skin involvement such as CD56+ AML, extranodal NK/T-cell lymphoma, and other T-cell lymphomas. Methods/Case Report A 71-year-old male presented to the emergency department with asymptomatic, pink- violaceous plaques/nodules on the trunk for 3 months. One month prior, a dermatologist diagnosed epidermal inclusion cysts that were left untreated. The nodules progressed and a course of steroids prescribed by a primary care physician provided short term improvement before the lesions flared again. Review of systems and vitals were normal. Biopsies of representative lesions sent for histologic examination showed a diffuse dermal infiltrate of small to medium atypical cells with irregular nuclear contours, fine chromatin, one to several nucleoli and scant cytoplasm. Immunohistochemistry showed these cells were positive for CD2, CD4, CD56 and CD45 with strong expression of BCL2 and focal CD123. The cells were negative for CD3, CD20, CD79a, CD8, CD30, ALK-1, MUM-1, CD10, Cyclin- D1, C-MYC, EBER, BCL6, Langerin, Granzyme, TIA1, CD68, CD163, MPO, and Lysozyme. The histology and immunoprofile were consistent with BPDCN. A bone marrow biopsy showed cells with similar morphology and staining pattern, including expression of CD123.Treatment with chemotherapy and Tagraxofusp, was initiated. Within a week, the patient showed near resolution of cutaneous lesions. Repeat bone marrow aspirate and flow cytometry a month later showed no malignant cells. Results (if a Case Study enter NA) NA Conclusion We present this case as a rare hematologic malignancy with a challenging clinical and histopathologic diagnosis. The histologic findings suggested either a high grade myeloid or lymphoid malignancy. The combination of CD3-/CD56+/CD4+/CD123+ in the lesional cells helped establish the diagnosis of BPDCN, allowing for prompt treatment.

scholarly journals Features of non-activation dendritic state and immune deficiency in blastic plasmacytoid dendritic cell neoplasm (BPDCN)

2019 ◽  
Vol 9 (12) ◽  
Author(s):  
Hannah C. Beird ◽  
Maliha Khan ◽  
Feng Wang ◽  
Mansour Alfayez ◽  
Tianyu Cai ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Dendritic Cell ◽  
Peripheral Blood ◽  
Profile Analysis ◽  
Hematologic Malignancy ◽  
Plasmacytoid Dendritic Cell ◽  
Serum Samples ◽  
Molecular Features ◽  
Cell Neoplasm ◽  
Poor Outcomes

AbstractBlastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, male-predominant hematologic malignancy with poor outcomes and with just one recently approved agent (tagraxofusp). It is characterized by the abnormal proliferation of precursor plasmacytoid dendritic cells (pDCs) with morphologic and molecular similarities to acute myeloid leukemia (AML) and myelodysplastic syndrome (MDS)/chronic myelomonocytic leukemia (CMML) in its presentation within the bone marrow and peripheral blood. To identify disease-specific molecular features of BPDCN, we profiled the bone marrow, peripheral blood, and serum samples from primary patient samples using an in-house hematologic malignancy panel (“T300” panel), transcriptome microarray, and serum multiplex immunoassays. TET2 mutations (5/8, 63%) were the most prevalent in our cohort. Using the transcriptome microarray, genes specific to pDCs (LAMP5, CCDC50) were more highly expressed in BPDCN than in AML specimens. Finally, the serum cytokine profile analysis showed significantly elevated levels of eosinophil chemoattractants eotaxin and RANTES in BPDCN as compared with AML. Along with the high levels of PTPRS and dendritic nature of the tumor cells, these findings suggest a possible pre-inflammatory context of this disease, in which BPDCN features nonactivated pDCs.

scholarly journals Comprehensive metagenomic analysis of blastic plasmacytoid dendritic cell neoplasm

2020 ◽  
Vol 4 (6) ◽  
pp. 1006-1011
Author(s):  
Jason Nomburg ◽  
Susan Bullman ◽  
Sun Sook Chung ◽  
Katsuhiro Togami ◽  
Mark A. Walker ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Dendritic Cell ◽  
Cell Carcinoma ◽  
Kaposi's Sarcoma ◽  
Hematologic Malignancy ◽  
Normal Skin ◽  
Plasmacytoid Dendritic Cell ◽  
Kaposi’S Sarcoma ◽  
Merkel Cell ◽  
Cell Neoplasm

Abstract Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a hematologic malignancy believed to originate from plasmacytoid dendritic cells (pDCs), the immune cells responsible for producing type 1 interferons during infection. Nearly all patients with BPDCN have prominent skin involvement, with cutaneous infiltration occupying the dermis and subcutis. One half of patients present with BPDCN cells only in the skin, with no evidence of disease elsewhere. Because normal pDCs are rare or absent in cutaneous sites, and they only traffic to the skin after activation by pathogen or inflammation, our aim was to determine if a microorganism is associated with BPDCN. We performed RNA sequencing in BPDCN skin and bone marrow, with cutaneous T-cell lymphoma (CTCL) and normal skin as controls. GATK-PathSeq was used to identify known microbial sequences. Bacterial reads in BPDCN skin were components of normal flora and did not distinguish BPDCN from controls. We then developed a new computational tool, virID (Viral Identification and Discovery; https://github.com/jnoms/virID), for identification of microbial-associated reads remaining unassigned after GATK-PathSeq. We found no evidence for a known or novel virus in BPDCN skin or bone marrow, despite confirming that virID could identify Merkel cell polyomavirus in Merkel cell carcinoma, human papillomavirus in head and neck squamous cell carcinoma, and Kaposi’s sarcoma herpesvirus in Kaposi’s sarcoma in a blinded fashion. Thus, at the level of sensitivity used here, we found no clear pathogen linked to BPDCN.

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.

scholarly journals Approval of tagraxofusp-erzs for blastic plasmacytoid dendritic cell neoplasm

2020 ◽  
Vol 4 (16) ◽  
pp. 4020-4027 ◽  
Author(s):  
Naveen Pemmaraju ◽  
Marina Konopleva
Keyword(s):  
Dendritic Cell ◽  
Hematologic Malignancy ◽  
Phase 1 ◽  
Plasmacytoid Dendritic Cell ◽  
Package Insert ◽  
Drug Approval ◽  
Clinical Aspects ◽  
Patients Âgés ◽  
Cell Neoplasm ◽  
Surface Receptor

Abstract Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and clinically challenging hematologic malignancy with dismal outcomes. With a median age of ∼70 years, the majority of patients with BPDCN have experienced historically suboptimal responses with intensive chemotherapy regimens. The major scientific breakthrough in this field was the recognition of overexpression of a surface receptor, CD123/interleukin 3 (IL-3) receptor α, in all patients. Importantly, a novel therapeutic agent consisting of a truncated diphtheria toxin (DT) payload fused to recombinant human IL-3 was being developed, one that targeted CD123, initially known as DT-IL-3 (later known as SL401; tagraxofusp; tagraxofusp-erzs [Elzonris]). The identification of this agent, and subsequent clinical trials specifically dedicated to patients with BPDCN (including a pilot study, followed by a larger phase 1/2 multicenter study [90% overall response rate [ORR] in frontline and 67% ORR in relapsed/refractory setting]), in part led to approval of tagraxofusp-erzs on 21 December 2018. Tagraxofusp-erzs was the first agent approved for BPDCN (for patients ages 2 years and older), and importantly, established this drug as the first CD123-targeted agent ever approved. The most notable toxicity of tagraxofusp-erzs is occurrence of the capillary leak syndrome, which occurs frequently at all grades, and has also been observed to be life-threatening, appropriately leading to a US Food and Drug Administration “black box” warning in the package insert. The preclinical and clinical aspects of drug development of tagraxofusp-erzs as monotherapy leading to drug approval are reviewed herein, with discussion of future directions of this novel agent, including consideration for rational combinations in BPDCN and beyond.

Clinical and Pathological Features and Differential Diagnosis of Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN): a Series of Cases

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 5183-5183 ◽  
Author(s):  
Gabriela Cesarman-Maus ◽  
Carmen Lome ◽  
Karla Adriana Espinosa ◽  
Carmen Marcela Quezada-Fiallos ◽  
Silvia Rivas ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Acute Myeloid Leukemia ◽  
Differential Diagnosis ◽  
Dendritic Cell ◽  
Myeloid Leukemia ◽  
Conflicts Of Interest ◽  
Plasmacytoid Dendritic Cell ◽  
Cell Neoplasm ◽  
Skin Infiltration ◽  
Acute Myeloid

Abstract Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) is a recently recognized highly aggressive malignant proliferation of plasmacytoid dendritic-cell (PDC) precursors which consistently express CD4, CD56 and CD123. Mortality is high despite transplant and the response to treatment is poor, except for preliminary results with conjugated anti-CD123. Clinically, cutaneous involvement is the most common feature with or without the presence of initial bone marrow infiltration, however patients may present with bone marrow-only disease. BPDCN is underdiagnosed, and can be confused with several entities, including acute myeloid leukemia. We describe our experience with 7 cases of BPDCN, their clinical and pathological presentation, and describe possible misdiagnosis and the antibodies that may help in corroborating BPDCN. See table for clinical characteristics at diagnosis. Diagnosis of BPDCN must take into account clinical, morphological and immunohistochemical analysis (IHC), since these tumors variably express markers that may be shared with other neoplasias including CD56 and TCL-1. When IHC is not categorical for BPDCN, the WHO recommends reporting the cases as AML of ambiguous lineage. The typical IHC includes CD4, CD43, CD45RA, CD56, CD123, TCL1, CLA and CD68. The absence of CD56 does not exclude the diagnosis. Markers shared with other hematological tumors include: CD7, CD33, CD2,CD36 and CD38 and TdT. Thus differential diagnosis should be done primarily with A) Skin infiltration by acute myeloid leukemia (myeloperoxidase +, 7- lysozyme +, CD34 +, CD117 +/-) B) Skin Infiltration by T / NK extra nodal lymphoma ( CD8 , cytotoxic cytoplasmic granules [CCG] , granzyme B, perforin, TIA1 and EBER) C) cutaneous peripheral T lymphoma ( +/- CD8, CD2 +/-, +/- CD5, CD7 +/- and variably positive CCG´s). D) Other histiocytic and dendritic cell-neoplasms may also be considered in the differential diagnosis however the histological appearance is usually characteristic. BPDCN is a poorly known entity that should be suspected by both clinician and pathologist in order to make a correct diagnosis. Table Table. Disclosures No relevant conflicts of interest to declare.

scholarly journals Pathology Quiz Case: Plasmacytoid Dendritic Cell Neoplasm

2014 ◽  
Vol 5 (1) ◽  
pp. ar.2014.5.0085 ◽  
Author(s):  
Quinn A. Dunlap ◽  
Kristine E. Day ◽  
Samuel G. Borak ◽  
Bradford A. Woodworth
Keyword(s):  
Dendritic Cell ◽  
Early Diagnosis ◽  
Hematologic Malignancy ◽  
Plasmacytoid Dendritic Cell ◽  
Current Case ◽  
Systemic Involvement ◽  
Cell Neoplasm ◽  
Putative Origin ◽  
Diagnostic Modalities ◽  
Paranasal Sinus Tumor

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy that possesses a heterogenous clinical and immunophenotypic presentation. The current case report describes an interesting and unique presentation of BPDCN as a primary paranasal sinus tumor without evidence of cutaneous or systemic involvement. As such, the report further contributes to the ongoing debate regarding the true putative origin of the neoplasm, as well as highlights the optimal diagnostic modalities, paramount importance of early diagnosis, and vast heterogeneity exhibited by this fascinating malignancy. The atypical presentation described here indicates the manifestations of BPDCN are more heterogenous than previously documented and thus can not be definitively ruled out in the absence of bone marrow, peripheral blood, or cutaneous involvement. Furthermore, atypical neoplastic presentations mandate flow cytometry and adjunctive immunohistochemistry for the definitive diagnosis of BPDCN, and early diagnosis of such neoplasms are critical for rapid initiation of treatment and improved outcomes.

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 Blastic plasmacytoid dendritic cell neoplasm with skin and bone marrow involvement: Report of three cases

2021 ◽  
Vol 9 (33) ◽  
pp. 10293-10299
Author(s):  
Jiang-Hong Guo ◽  
Hong-Wei Zhang ◽  
Li Wang ◽  
Wei Bai ◽  
Jin-Fen Wang
Keyword(s):  
Bone Marrow ◽  
Dendritic Cell ◽  
Plasmacytoid Dendritic Cell ◽  
Bone Marrow Involvement ◽  
Cell Neoplasm

scholarly journals An Unusual Manifestation of Blastic Plasmacytoid Dendritic Cell Neoplasm as a Testicular Tumor

2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Daniel M. Pak ◽  
Maria S. Tretiakova
Keyword(s):  
Dendritic Cell ◽  
Hematologic Malignancy ◽  
Plasmacytoid Dendritic Cell ◽  
Testicular Tumor ◽  
Integrated Approach ◽  
Significant Heterogeneity ◽  
Tumor Pathology ◽  
First Case ◽  
Cell Neoplasm ◽  
Immunohistochemical Studies

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a clinically aggressive hematologic malignancy arising from precursors of plasmacytoid dendritic cells that represent less than 1% of hematological malignancies. BPDCN initially presents with cutaneous involvement and a characteristic immunophenotype of CD4, CD56, and CD123 co-expression. Upon disease progression, BPDCN shows a strong predilection for bone marrow, peripheral blood, and lymph nodes, whereas manifestations in visceral organs are rare. Significant heterogeneity in clinical presentation and immunophenotypic profile makes BPDCN challenging to diagnose without an integrated approach based on patient history, clinical features, tumor pathology, and comprehensive immunohistochemical studies. Herein we report the first case of relapsed BPDCN manifesting as a unilateral testicular tumor.

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