U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

CD4+/CD56+ hematodermic neoplasm(BPDCN)

MedGen UID:
220972
Concept ID:
C1301363
Neoplastic Process
Synonyms: Blastic plasmacytoid dendritic cell; Blastic plasmacytoid dendritic cell neoplasm; BPDCN
SNOMED CT: Blastic plasmacytoid dendritic cell neoplasm (445030005); Blastic plasmacytoid dendritic cell neoplasm (445105005)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0019467
Orphanet: ORPHA86870

Definition

A rare hematologic neoplasm characterized by origin from precursors of plasmacytoid dendritic cells, with frequent cutaneous, bone marrow, and lymph node involvement, as well as leukemic dissemination. Most common clinical presentation is with asymptomatic solitary or multiple skin lesions (either isolated purplish nodules, isolated bruise-like papules, or disseminated purplish nodules/macules/papules), although some patients may present with leukemia. Skin biopsy shows a diffuse, monomorphous infiltrate of medium-sized blast cells resembling either lymphoblasts or myeloblasts, with massive involvement of the dermis. The clinical course is aggressive, and age has an adverse impact on prognosis. [from ORDO]

Recent clinical studies

Etiology

Zindanci I, Kavala M, Buyukbabani N, Kocaturk E, Koc M
Indian J Dermatol Venereol Leprol 2010 Nov-Dec;76(6):723. doi: 10.4103/0378-6323.72471. PMID: 21079332
Wang P, Wang Z, Kong Y, Xu A
Int J Dermatol 2009 Jan;48(1):62-5. doi: 10.1111/j.1365-4632.2009.03777.x. PMID: 19126053
Liang X, Graham DK
Cancer 2008 Apr 1;112(7):1425-36. doi: 10.1002/cncr.23316. PMID: 18286525
Pilichowska ME, Fleming MD, Pinkus JL, Pinkus GS
Am J Clin Pathol 2007 Sep;128(3):445-53. doi: 10.1309/W9Q5AGYDE5LANN39. PMID: 17709319
Petrella T, Bagot M, Willemze R, Beylot-Barry M, Vergier B, Delaunay M, Meijer CJ, Courville P, Joly P, Grange F, De Muret A, Machet L, Dompmartin A, Bosq J, Durlach A, Bernard P, Dalac S, Dechelotte P, D'Incan M, Wechsler J, Teitell MA
Am J Clin Pathol 2005 May;123(5):662-75. PMID: 15981806

Diagnosis

Zindanci I, Kavala M, Buyukbabani N, Kocaturk E, Koc M
Indian J Dermatol Venereol Leprol 2010 Nov-Dec;76(6):723. doi: 10.4103/0378-6323.72471. PMID: 21079332
Hu SC, Tsai KB, Chen GS, Chen PH
Haematologica 2007 Sep;92(9):e91-3. doi: 10.3324/haematol.11307. PMID: 17768139
Herling M, Jones D
Am J Clin Pathol 2007 May;127(5):687-700. doi: 10.1309/FY6PK436NBK0RYD4. PMID: 17439829
Niakosari F, Sur M
Arch Pathol Lab Med 2007 Jan;131(1):149-51. doi: 10.5858/2007-131-149-ACHNAD. PMID: 17227117
Petrella T, Bagot M, Willemze R, Beylot-Barry M, Vergier B, Delaunay M, Meijer CJ, Courville P, Joly P, Grange F, De Muret A, Machet L, Dompmartin A, Bosq J, Durlach A, Bernard P, Dalac S, Dechelotte P, D'Incan M, Wechsler J, Teitell MA
Am J Clin Pathol 2005 May;123(5):662-75. PMID: 15981806

Therapy

Su O, Onsun N, Demirkesen C, Aydin Y, Pirmit S, Gereli M
Dermatol Online J 2010 Apr 15;16(4):8. PMID: 20409415
Shiman M, Marchione R, Ricotti C, Romanelli P, Alonso-Llamazares J
Dermatol Online J 2008 Nov 15;14(11):5. PMID: 19094843
Ascani S, Massone C, Ferrara G, Rongioletti F, Papini M, Pileri S, Cerroni L
J Cutan Pathol 2008 Oct;35(10):911-5. Epub 2008 May 20 doi: 10.1111/j.1600-0560.2007.00915.x. PMID: 18494823
Stetsenko GY, McFarlane R, Kalus A, Olerud J, Cherian S, Fromm J, George E, Argenyi Z
J Cutan Pathol 2008 Jun;35(6):579-84. Epub 2007 Nov 12 doi: 10.1111/j.1600-0560.2007.00845.x. PMID: 18005171
Ruggiero A, Maurizi P, Larocca LM, Arlotta A, Riccardi R
Haematologica 2006 Dec;91(12 Suppl):ECR48. PMID: 17194654

Prognosis

Zindanci I, Kavala M, Buyukbabani N, Kocaturk E, Koc M
Indian J Dermatol Venereol Leprol 2010 Nov-Dec;76(6):723. doi: 10.4103/0378-6323.72471. PMID: 21079332
Su O, Onsun N, Demirkesen C, Aydin Y, Pirmit S, Gereli M
Dermatol Online J 2010 Apr 15;16(4):8. PMID: 20409415
Hu SC, Tsai KB, Chen GS, Chen PH
Haematologica 2007 Sep;92(9):e91-3. doi: 10.3324/haematol.11307. PMID: 17768139
Herling M, Jones D
Am J Clin Pathol 2007 May;127(5):687-700. doi: 10.1309/FY6PK436NBK0RYD4. PMID: 17439829
Niakosari F, Sur M
Arch Pathol Lab Med 2007 Jan;131(1):149-51. doi: 10.5858/2007-131-149-ACHNAD. PMID: 17227117

Clinical prediction guides

Zheng G, Schmieg J, Guan H, Ali SZ
Acta Cytol 2012;56(2):204-8. Epub 2012 Feb 17 doi: 10.1159/000332365. PMID: 22378086
Eros N, Marschalkó M, Balassa K, Hídvégi B, Szakonyi J, Ilniczky S, Borka K, Kovács A, Bottlik G, Hársing J, Csomor J, Szepesi A, Matolcsy A, Kárpáti S, Demeter J
J Neurooncol 2010 Apr;97(2):301-4. Epub 2009 Oct 2 doi: 10.1007/s11060-009-0026-y. PMID: 19798469
Jaye DL, Geigerman CM, Herling M, Eastburn K, Waller EK, Jones D
Mod Pathol 2006 Dec;19(12):1555-62. Epub 2006 Sep 22 doi: 10.1038/modpathol.3800679. PMID: 16998465

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    Curated

    • NCCN, 2023
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Acute Myeloid Leukemia, 2023

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...