lschriml
commented
2 years ago Notes:
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
A clinically aggressive neoplasm derived from the precursors of plasmacytoid dendritic cells (also called professional type I interferon-producing cells or plasmacytoid monocytes), with a high frequency of cutaneous and bone marrow involvement and leukemic dissemination. (WHO 2017)
synonyms: Agranular CD4+ CD56+ Hematodermic Neoplasm/Tumor CD4+/CD56+ Hematodermic Neoplasm
Antiquated synonyms:
Agranular CD4+ Natural Killer Cell Leukemia
Blastic Natural Killer Leukemia/Lymphoma
Blastic NK-Cell Lymphoma
https://www.lls.org/leukemia/blastic-plasmacytoid-dendritic-cell-neoplasm Blastic plasmacytoid dendritic cell neoplasm (BPDCN) was previously known as natural killer (NK) cell leukemia/lymphoma.
https://www.mdanderson.org/cancer-types/leukemia.html?invsrc=leukemia&cmpid=LUK_ORG_leukemia_G_SE&gclid=CjwKCAjwp7eUBhBeEiwAZbHwkZsg5_-eGdZRKv2gn7ODeQt8Vv9Fxj16VVgg5pXgMuXKj_jafjLVbBoCgekQAvD_BwE&gclsrc=aw.ds MD Anderson: BPDCN is an aggressive subtype of acute leukemia that impacts approximately 500 to 1,000 people in the U.S. each year. Though it can affect people of any age, the disease is most common among older patients, with about half of new diagnoses coming in patients age 70 or older. Historically, it has been associated with poor survival rates.
BPDCN is a cancer of a specific type of cell known as plasmacytoid dendritic cells. This rare type of immune system cell is usually found in the skin, bone marrow, blood, lymph nodes and spleen. As a result, symptoms usually can impact these areas. Skin lesions are the most common BPDCN symptom. These can show up anywhere on the body but often appear on the arms, legs, face and neck. Other symptoms include low counts of healthy blood cells and swollen lymph nodes.
Traditionally, BPDCN has been treated with chemotherapy, while younger, fitter patients have been given the option of a stem cell transplant. The Food and Drug Administration has recently approved a new targeted therapy drug that attacks CD123, a molecule commonly found on the surface of BPDCN cells. This targeted therapy is now considered a standard treatment for the disease. Learn more about BPDCN.
Received this request via email. From: taofei.yin@jax.org Would you consider adding Blastic plasmacytoid dendritic cell neoplasm to the DO terms?