A patient's face with one side drifting into purple blood cells representing a hematologic neoplasmA patient's face with one side drifting into purple blood cells representing a hematologic neoplasm
Dermatologists are
the first line of defense
against BPDCN1,2
Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN)
Quick look at BPDCN
  • BPDCN is an aggressive and deadly blood cancer that often presents with skin lesions3,4
  • An early skin biopsy can help patients obtain an accurate and timely diagnosis of their hematologic malignancies1,5
  • Cooperation with pathologists and hemato-oncologists is key for a correct diagnosis6
  • Discuss CD123 testing with your pathologist
An early skin biopsy can aid accurate and timely diagnosis of hematologic malignancies1
If you see a patient with unusual or treatment-resistant skin lesions, consider a biopsy7,8
Innocent skin lesion or deadly hematologic disease?
The dermatologist’s judgment is critical9
Would you suspect a hematologic malignancy?
Violet-to-purple
nodules10 
Violet-to-purple skin noduleA
Courtesy of Shapiro R, et al. J Cell Sci Ther. S8:008. doi: 10.4172/2157-7013.S8-008.8.
“Bruise-like”
infiltrates7 
Bruise-like infiltrates B
Images republished with permission from SAGE Publications.
Hyperpigmented
red-brown macules11
Hyperpigmented red-brown maculesC
Images republished with permission from the American Society of Hematology
A: Courtesy of Shapiro R, et al. J Cell Sci Ther. S8:008. doi: 10.4172/2157-7013.S8-008.8. B: Images republished with permission from SAGE Publications. C: Images republished with permission from the American Society of Hematology
Rapid detection of blood cancers is critical5
Patients with hematologic malignancies frequently have a poor prognosis.
One rare and aggressive hematologic malignancy that presents with skin lesions is BPDCN3,6,12
What is BPDCN?
BPDCN is an aggressive and deadly blood cancer that often presents with skin lesions3,4
  • Average time between onset of lesions and diagnosis of BPDCN is 6 months3,12
  • Historically, median overall survival is approximately 8 to 14 months after diagnosis3,12
Historical overall survival rate for patients diagnosed with BPDCN
BPDCN presentation:
 
~85% to 90%
of patients present with skin lesions, which can vary in size, shape and color3,7,10,13
 
~75%
of patients are men7,12
Typically patients are
60 to 70 years
of age, but BPDCN can affect people of any age5,7,12
Cutaneous manifestations may present as3,7,13
  • Deep purple nodular lesions, localized to various body areas, particularly the trunk, limbs, and head
  • Diffuse bruise-like or hyperpigmented red-brown macules
    – may be small and/or diffuse
    – one or several patches confined to various body areas
    – often nonpruritic
  • Disseminated and mixed lesions
  • A deep tissue sample should be provided for evaluation, as BPDCN typically spares the epidermis4,7,10
Nodular lesions
Nodular lesions
Nodular lesion caused by BPDCNA
Images republished with permission: This is an electronic version of a figure published in The Oncologist, December 2009, published by Wiley.
Nodular lesion caused by BPDCNB
Images republished with permission from Springer Nature: Modern Pathology, Facchetti, F, © 2016.
Facial nodular lesion caused by BPDCNC
Images republished with permission from Elsevier.
A: Images republished with permission: This is an electronic version of a figure published in The Oncologist, December 2009, published by Wiley.
B: Images republished with permission from Springer Nature: Modern Pathology, Facchetti, F, © 2016.
C: Images republished with permission from Elsevier.
Bruise-like macules or hyperpigmented presentations
Bruise-like macules or hyperpigmented presentations
Bruise-like macules caused by BPDCNA
Images republished with permission from the Massachusetts Medical Society.
Hyperpigmented skin lesions caused by BPDCNB
Images republished with permission from Julia F, et al., Br J Dermatol. 2013;169:579-586, published by John Wiley and Sons. ©2013 British Association of Dermatologists
Bruise-like lesions caused by BPDCNC
Reprinted with permission from Blood.
A: Images republished with permission from the Massachusetts Medical Society. B: Images republished with permission from Julia F, et al., Br J Dermatol. 2013;169:579-586, published by John Wiley and Sons. ©2013 British Association of Dermatologists
C: Reprinted with permission from Blood.
CD123 is a diagnostic marker that can help to identify BPDCN*4,5,14
* The diagnosis of BPDCN requires immunophenotyping and fundamentally relies on the demonstration of CD4, CD56, CD123, CD303, and TCL1 expression, together with a lack of expression of markers for B cells, T cells, myeloid or monocytic cells, and NK cells.4,5
How to diagnose BPDCN?
Close cooperation with pathologists and hemato-oncologists is key for a correct diagnosis6
  • While ~85% to 90% of patients present with skin lesions, ~60% to 90% of patients present with leukemic disease3,7,10,13,15
  • Recognizing unusual or treatment-resistant skin lesions, promptly testing them, and referring patients to hemato-oncologists will help patients receive an accurate diagnosis quicker
Multidisciplinary cooperation reducing the lapse between lesion presentation and diagnosis may improve patient outcomes.6
Discuss CD123 testing with your pathologist
  • CD123 is very important in helping to identify and differentiate hematologic malignancies16
  • The marker triad – CD123, CD4, and CD56 – can help confirm the diagnosis of BPDCN*5,12-14,17
    – CD123 is highly expressed on BPDCN cells (~ 95 %) and negligibly expressed on healthy cells2,12,14,17,18
    – Can be identified through any biopsy of malignant cells4,16
A graphic showing that BPDCN testing should include CD123, in combination with CD4 and CD56
* The diagnosis of BPDCN requires immunophenotyping and fundamentally relies on the demonstration of CD4, CD56, CD123, CD303, and TCL1 expression, together with lack of expression of markers for B cells, T cells, myeloid or monocytic cells, and NK cells.4,5
Early skin biopsy can aid accurate and timely diagnosis of hematologic malignancies1,5
Time is all-important for patients with hematologic malignancies
If you see a patient with unusual or treatment-resistant skin lesions, consider a biopsy7,8
Abbreviations
BPDCN= blastic plasmacytoid dendritic cell neoplasm;
CD = cluster of differentiation;
NK = natural killer;
TCL1 = T-cell leukemia/lymphoma protein 1A.
References
  1. References:
  2. 1. Hirner JP, et al. Hematol Oncol Clin North Am. 2020 Jun;34(3):501-509.
  3. 2. Deconinck E, et al. Hematol Oncol Clin North Am. 2020;34(3):491-500.
  4. 3. Julia F, et al. Br J Dermatol. 2013;169(3):579-586.
  5. 4. Facchetti F, et al. Mod Pathol. 2016;29(2):98-111.
  6. 5. Pagano L, et al. Br J Haematol. 2016;174(2):188-202.
  7. 6. Deconinck E, et al. 2021 Fast Facts© S. Karger Publishers Ltd.
  8. 7. Riaz W, et al. Cancer Control. 2014;21(4):279-289.
  9. 8. Alguire PC, Mathes BM. UpToDate. Official Reprint. Updated May 10, 2021. Accessed Sept 24, 2021
  10. 9. Fogo A, du Vivier A. Clin Med (Lond). 2009 Aug;9(4):366-70.
  11. 10. Shapiro R, et al. J Cell Sci Ther. 2015;S8:008.
  12. 11. Sullivan JM, Rizzieri DA. Hematology Am Soc Hematol Educ Program. 2016;2016(1):16-23.
  13. 12. Pagano L, et al. Haematologica. 2013;98(2):239-246.
  14. 13. Laribi K, et al. Biol Blood Marrow Transplant. 2016;22(8):1357-1367.
  15. 14. Pemmaraju N, Konopleva M. Blood Adv. 2020 Aug 25;4(16):4020-4027.
  16. 15. Herling M, Jones D. Am J Clin Pathol. 2007 May;127(5):687-700.
  17. 16. Patnaik MM, et al. Leuk Lymphoma. 2021 Nov;62(11):2568-2586.
  18. 17. Frankel AE, et al. Blood. 2014;124(3):385-392.
  19. 18. Pemmaraju N. Curr Hematol Malig Rep. 2017;12(6):510-512.