Blastic plasmacytoid dendritic cell neoplasm

Blastic plasmacytoid dendritic cell neoplasm
CD4+ CD56+ lymphoma

Blastic plasmacytoid dendritic cell neoplasm (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,[1] and agranular CD4+ NK cell leukemia.[2] Later, however, the disease was determined to be a malignancy of plasmacytoid dendritic cells rather than lymphocytes and therefore termed blastic plasmacytoid dendritic cell neoplasm. In 2016, the World Health Organization designated BPDCN to be in its own separate category within the myeloid class of neoplasms.[3] It is estimated that BPDCN constitutes 0.44% of all hematological malignancies.[4]

Blastic plasmacytoid dendritic cell neoplasm is an aggressive malignancy with features of cutaneous lymphoma (e.g. malignant plasmacytoid dendritic cell infiltrations into the skin to form single or multiple lesions) and/or leukemia (i.e. malignant plasmacytoid dendritic cells in blood and bone marrow).[2] While commonly presenting with these clinical features, BPDCN, particularly in its more advanced stages, may also involve malignant plasmacytoid dendritic cell infiltrations in and thereby injury to the liver, spleen, lymph nodes, central nervous system, or other tissues. The neoplasm 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.[5]

Blastic plasmacytoid dendritic cell neoplasm 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.[5] Furthermore, the disease may occur in association with the myelodysplastic syndrome or transform to acute myeloid leukemia.[4] Consequently, BPDCN has a very low 5 year survival rate.[5] Current translational research studies on treating BPDCN have therefore focused on non-chemotherapeutic regimens that target the molecular pathways which may promote the disease.[6]

  1. ^ Slater DN (November 2005). "The new World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas: a practical marriage of two giants". The British Journal of Dermatology. 153 (5): 874–80. doi:10.1111/j.1365-2133.2005.06905.x. PMID 16225594. S2CID 23625855.
  2. ^ a b Owczarczyk-Saczonek A, Sokołowska-Wojdyło M, Olszewska B, Malek M, Znajewska-Pander A, Kowalczyk A, Biernat W, Poniatowska-Broniek G, Knopińska-Posłuszny W, Kozielec Z, Nowicki R, Placek W (April 2018). "Clinicopathologic retrospective analysis of blastic plasmacytoid dendritic cell neoplasms". Postepy Dermatologii I Alergologii. 35 (2): 128–138. doi:10.5114/ada.2017.72269. PMC 5949541. PMID 29760611.
  3. ^ Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, Bloomfield CD, Cazzola M, Vardiman JW (May 2016). "The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia". Blood. 127 (20): 2391–405. doi:10.1182/blood-2016-03-643544. PMID 27069254.
  4. ^ a b Sullivan JM, Rizzieri DA (December 2016). "Treatment of blastic plasmacytoid dendritic cell neoplasm". Hematology. American Society of Hematology. Education Program. 2016 (1): 16–23. doi:10.1182/asheducation-2016.1.16. PMC 6142460. PMID 27913457.
  5. ^ a b c Kim MJ, Nasr A, Kabir B, de Nanassy J, Tang K, Menzies-Toman D, Johnston D, El Demellawy D (October 2017). "Pediatric Blastic Plasmacytoid Dendritic Cell Neoplasm: A Systematic Literature Review". Journal of Pediatric Hematology/Oncology. 39 (7): 528–537. doi:10.1097/MPH.0000000000000964. PMID 28906324. S2CID 11799428.
  6. ^ Pemmaraju N (December 2017). "Novel Pathways and Potential Therapeutic Strategies for Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN): CD123 and Beyond". Current Hematologic Malignancy Reports. 12 (6): 510–512. doi:10.1007/s11899-017-0425-7. PMID 29064022. S2CID 207330989.