Table of Contents
Where are plasmacytoid dendritic cells?
peripheral lymphoid organs
Plasmacytoid dendritic cells (pDCs) are a rare type of immune cell that are known to secrete large quantities of type 1 interferon (IFNs) in response to a viral infection. They circulate in the blood and are found in peripheral lymphoid organs.
What is plasmacytoid dendritic cell neoplasm?
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, clinically aggressive hematologic malignancy that most commonly manifests as cutaneous lesions with or without bone marrow involvement and leukemic dissemination.
How is BPDCN diagnosed?
80%–90% of patients with BPDCN present with skin lesions. Early recognition can lead to timely diagnosis and management. Accurate diagnosis requires a biopsy showing the morphology of plasmacytoid dendritic blast cells and immunophenotypic criteria established by either immunohistochemistry or flow cytometry.
What is the meaning of plasmacytoid?
Medical Definition of plasmacytoid : resembling or derived from a plasma cell.
What is the function of plasmacytoid dendritic cells?
Plasmacytoid dendritic cells (pDCs) are a unique subset of dendritic cells specialised in secreting high levels of type I interferons. pDCs play a crucial role in antiviral immunity and have been implicated in the initiation and development of many autoimmune and inflammatory diseases.
What activates plasmacytoid dendritic cells?
Plasmacytoid dendritic cells (pDC) are activated indirectly or directly by immune complexes and secrete high amounts of type I interferon in a TLR7 and/or 9-dependent mechanism.
Who blastic plasmacytoid dendritic cell neoplasm?
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) was previously known as natural killer (NK) cell leukemia/lymphoma. As understanding of the biology and origin of this malignancy has improved, the World Health Organization (WHO) established the term blastic plasmacytoid dendritic cell neoplasm (BPDCN) in 2008.
Is BPDCN a form of AML?
BPDCN was initially classified within the acute myeloid leukemia (AML)–related precursor neoplasms in the 2008 World Health Organization classification,1 although it was reclassified as a separate entity in the recently revised edition.
Is BPDCN AML?
What is the major trigger for development of plasmacytoid dendritic cells?
Although macrophages and some dendritic cells (DCs) reside in the arterial wall, increased influx of extravasated monocytes is thought to be the major trigger of inducing the inflammatory response in affected intima media (Ley et al., 2011).
Are pDCs myeloid cells?
Plasmacytoid dendritic cells (pDCs) can be derived from both myeloid and lymphoid progenitors.
What causes blastic plasmacytoid dendritic cell neoplasm?
It mainly affects older patients, but can affect people of any age. Blastic plasmacytoid dendritic cell neoplasm springs from a rare immune cell that is usually found in the skin, bone marrow, blood, lymph nodes and spleen.
What is leukemia cutis?
Leukaemia cutis is a rare condition in which leukaemia cells are found in the skin tissue. This infiltration of the skin causes visible patches or lesions to appear which can be a distinctive sign of a person having leukaemia.
Is blastic plasmacytoid dendritic cell neoplasm a leukemia?
Naveen Pemmaraju, M.D., led the clinical trial for a new BPDCN treatment. Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare type of acute leukemia that impacts about 500-1,000 people in the U.S. each year. It mainly affects older patients, but can affect people of any age.
Where does leukemia cutis start?
While the legs are the most common area for leukemia cutis lesions to appear, they can also form on the arms, face, trunk, and scalp. These skin changes can include: papules, or raised, tender bumps on the skin’s surface. nodules, or lumps that form under the skin.
What are plasmacytoid dendritic cells?
Plasmacytoid dendritic cells (pDCs) are a unique sentinel cell type that can detect pathogen-derived nucleic acids and respond with rapid and massive production of type I interferon.
How do human plasmacytoid dendritic cells elicit a type I interferon response?
Bode C, Fox M, Tewary P et al Human plasmacytoid dendritic cells elicit a Type I Interferon response by sensing DNA via the cGAS‐STING signaling pathway. Eur J Immunol 2016; 46: 1615–1621.
Which plasmacytoid dendritic cells are dispensable for inflammatory bowel disease (IBD)?
Sawai C, Serpas L, Neto A et al Plasmacytoid dendritic cells are largely dispensable for the pathogenesis of experimental inflammatory bowel disease. Front Immunol 2018; 9: 1–16. [ PMC free article] [ PubMed] [ Google Scholar]
What regulates dynamic accumulation of plasmacytoid dendritic cells in lymph nodes?
Dynamic accumulation of plasmacytoid dendritic cells in lymph nodes is regulated by IFN‐β. Blood 2009; 114: 2623–2632. [ PubMed] [ Google Scholar] 49. Srivatsan S, Swiecki M, Otero K, Cella M, Shaw AS. CD2‐associated protein regulates plasmacytoid dendritic cell migration, but is dispensable for their development and cytokine production.