<unk>  (BPDCN) is a rare hematologic malignancy. It was initially regarded as a form of lymphocyte-derived cutaneous lymphoma and alternatively named CD4+CD56+ hematodermic tumor, blastic NK cell lymphoma, and agranular CD4+ NK cell leukemia. Later, however, the disease was determined to be a malignancy of<unk><unk>  cells rather than lymphocytes and therefore termed<unk> . In 2016, the World Health Organization designated BPDCN to be in its own separate category within the myeloid class of<unk> s. It is estimated that BPDCN constitutes 0.44% of all hematological malignancies.<unk>  is an aggressive malignancy with features of cutaneous lymphoma (e.g. malignant<unk><unk>  cell infiltrations into the skin to form single or multiple lesions) and/or leukemia (i.e. malignant<unk><unk>  cells in blood and bone marrow). While commonly presenting with these clinical features, BPDCN, particularly in its more advanced stages, may also involve malignant<unk><unk>  cell infiltrations in and thereby injury to the liver, spleen, lymph nodes, central nervous system, or other tissues. The<unk>  occurs in individuals of all ages but predominates in the elderly; in children, it afflicts males and females equally but in adults is far more common (~75% of cases) in males.<unk>  typically responds to chemotherapy regimens used to treat hematological malignancies. All too often, however, the disease rapidly recurs and does so in a more drug-resistant form. Furthermore, the disease may occur in association with the myelodysplastic syndrome or transform to acute myeloid leukemia. Consequently, BPDCN has a very low 5 year survival rate. Current translational research studies on treating BPDCN have therefore focused on non-chemotherapeutic regimens that target the molecular pathways which may promote the disease.
Blastic plasmacytoid dendritic cell neoplasm
Congenital self-healing reticulohistiocytosis
Nodal marginal zone B cell lymphoma
Angioimmunoblastic T-cell lymphoma
Mature T-cell lymphoma
Peripheral T-cell lymphoma
Precursor T-lymphoblastic lymphoma
Pleomorphic T-cell lymphoma