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Early diagnosis may be critical and requires a multidisciplinary team1

  • Dermatologists may be the first to recognize the signs of this hematologic malignancy because ~85% to 90% of patients present with skin lesions1-4
  • Pathologists are essential in diagnosing BPDCN, as correct diagnosis relies on a compatible immunophenotype, including the signature marker triad, CD123, CD4, and CD56, among other markers1,5*
  • Hematologist-oncologists are key in accurately diagnosing BPDCN, differentiating it from more common cancers with similar presentations1,4,6,7

Historically, BPDCN has had poor clinical outcomes8,9

Historical overall survival8

  • Historically, median overall survival for BPDCN is approximately 8 to 14 months after diagnosis8,9
  • In a retrospective analysis, the mean time between the onset of lesions and the final diagnosis of BPDCN was 6.2 months2

BPDCN is currently recognized as a unique myeloid neoplasm after years of reclassification

Evolving World Health Organization classification for BPDCN

2001

Blastic NK-cell lymphoma10

2005

CD4+ CD56+ hematodermic neoplasm10,11

2008

BPDCN as a subset of AML12

2016

BPDCN as a unique myeloid neoplasm13

NK = natural killer; AML = acute myeloid leukemia.

Frequent reclassification and renaming have likely contributed to the underrecognition of BPDCN1

Who are patients with BPDCN?

The exact incidence of BPDCN is unknown because the nomenclature used to describe BPDCN has evolved over the years along with an understanding of the underlying biology.1

75%

are men14

Typically between

60 to 70

years of age,

but all ages can be affected1

Affects all races and
geographic locations1

Have you seen a case of BPDCN?

Similarity to other hematologic malignancies may contribute to misdiagnosis.1,4,7,13 Approximately 10% to 20% of patients with BPDCN have a previous history of, or may have been misdiagnosed with, certain other hematologic malignancies, including MDS, CMML, and AML.1

BPDCN may be mistaken for

  • Acute myeloid leukemia (AML)
  • Leukemia cutis
  • Myeloid sarcoma
  • NK/T-cell lymphoma
  • Acute lymphoblastic leukemia (ALL)
  • Myelodysplastic syndrome (MDS)
  • Chronic myelomonocytic leukemia (CMML)
  • Cutaneous T-cell lymphoma (CTCL)

To help identify BPDCN, think CD1234561,5

  • CD123, CD4, and CD56 comprise a signature marker triad that is key in diagnosing BPDCN1,5*
  • CD123 can be both a diagnostic marker and a therapeutic target in BPDCN4,9,15
  • BPDCN can be diagnosed through any biopsy of malignant cells15

*BPDCN diagnosis can include other markers, such as TCL1, TCF4, and CD303 (BDCA2).1,16

Delay in the correct diagnosis can mean that by the time BPDCN is recognized, disease progression may already have occurred1,6,8
Find out about BPDCN’s variable dermatologic presentation
  1. References:
  2. Pagano L, et al. Blastic plasmacytoid dendritic cell neoplasm: diagnostic criteria and therapeutical approaches. Br J Haematol. 2016;174(2):188-202.
  3. Julia F, et al. Blastic plasmacytoid dendritic cell neoplasm: clinical features in 90 patients. Br J Dermatol. 2013;169(3):579-586.
  4. Sullivan JM, Rizzieri DA. Treatment of blastic plasmacytoid dendritic cell neoplasm. Hematology Am Soc Hematol Educ Program. 2016;2016(1):16-23.
  5. Laribi K, et al. Blastic plasmacytoid dendritic cell neoplasm: from origin of the cell to targeted therapies. Biol Blood Marrow Transplant. 2016;22(8):1357-1367.
  6. Pemmaraju N, Konopleva M. Treating blastic plasmacytoid dendritic cell neoplasm. The Hematologist website. http://www.hematology.org/Thehematologist/Ask/8927.aspx. Published August 28, 2018. Accessed April 25, 2019.
  7. Riaz W, et al. Blastic plasmacytoid dendritic cell neoplasm: update on molecular biology, diagnosis, and therapy. Cancer Control. 2014;21(4):279-289.
  8. Goyal A, et al. Blastic plasmacytoid dendritic cell neoplasm. In: Carter JB, et al, eds. Atlas of Cutaneous Lymphomas: Classification and Differential Diagnosis. Cham, Switzerland: Springer International; 2015:193-203.
  9. Pagano L, et al. Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study. Haematologica. 2013;98(2):239-246.
  10. Pemmaraju N. Novel pathways and potential therapeutic strategies for blastic plasmacytoid dendritic cell neoplasm (BPDCN): CD123 and beyond. Curr Hematol Malig Rep. 2017;12(6):510-512.
  11. Chan JK, et al. Blastic NK-cell lymphoma. In: Jaffe ES, et al, eds. WHO Classification. Tumours of Haematopoietic and Lymphoid Tissues. Lyon, France: IARC Press; 2001:214-215.
  12. Willemze R, et al. WHO-EORTC classification for cutaneous lymphomas. Blood. 2005;105(10):3768-3785.
  13. Facchetti F, et al. Blastic plasmacytoid dendritic cell neoplasm. In: Swerdlow SH, et al, eds. WHO Classification of Tumours. Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues. 4th ed. Lyon, France: IARC Press; 2008:145-147.
  14. Arber DA, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016; 127(20):2391-2405.
  15. Togami K, et al. Sex-biased ZRSR2 mutations in myeloid malignancies impair plasmacytoid dendritic cell activation and apoptosis. doi:10.1101/2020.10.29.36503.
  16. Facchetti F, et al. Neoplasms derived from plasmacytoid dendritic cells. Mod Pathol. 2016;29(2):98-111.
  17. Ceribelli M, et al. A druggable TCF4- and BRD4-dependent transcriptional network sustains malignancy in blastic plasmacytoid dendritic cell neoplasm. Cancer Cell. 2016;30(5):764-778.

INDICATION

  • ELZONRIS is a CD123-directed cytotoxin for the treatment of blastic plasmacytoid dendritic cell neoplasm (BPDCN) in adults and in pediatric patients 2 years and older

IMPORTANT SAFETY INFORMATION

Boxed WARNING: CAPILLARY LEAK SYNDROME

  • Capillary Leak Syndrome (CLS), which may be life-threatening or fatal, can occur in patients receiving ELZONRIS. Monitor for signs and symptoms of CLS and take actions as recommended

WARNINGS AND PRECAUTIONS

Capillary Leak Syndrome

  • ELZONRIS can cause capillary leak syndrome (CLS), which may be life-threatening or fatal if not properly managed. The overall incidence of CLS in clinical trials was 55% in patients receiving ELZONRIS, including 46% in Grades 1 or 2, 6% in Grade 3, 1% in Grade 4, and 2 fatal events. Common signs and symptoms (incidence ≥ 20%) associated with CLS that were reported during treatment with ELZONRIS include hypoalbuminemia, edema, weight gain, and hypotension
  • Before initiating therapy with ELZONRIS, ensure that the patient has adequate cardiac function and serum albumin is ≥ 3.2 g/dL
  • During treatment with ELZONRIS, ensure that serum albumin levels are ≥ 3.5 g/dL and have not been reduced by ≥ 0.5 g/dL from the albumin value measured prior to dosing initiation of the current cycle. Monitor serum albumin levels prior to the initiation of each dose or more often as indicated clinically thereafter. Additionally, assess patients for other signs or symptoms of CLS, including weight gain, new onset or worsening edema including pulmonary edema, hypotension, or hemodynamic instability
  • Counsel patients to seek immediate medical attention should signs or symptoms of CLS occur at any time

Hypersensitivity Reactions

  • ELZONRIS can cause severe hypersensitivity reactions. Grade 3 or higher events were reported in 10% of patients in clinical trials. Monitor patients for hypersensitivity reactions during treatment with ELZONRIS. Interrupt ELZONRIS infusion and provide supportive care as needed if a hypersensitivity reaction should occur. If the reaction is severe, discontinue ELZONRIS permanently

Hepatotoxicity

  • Elevations in liver enzymes can occur with ELZONRIS. Grade 3 or higher elevations in liver enzymes occurred in approximately 40% of patients in clinical trials
  • Monitor alanine aminotransferase (ALT) and aspartate aminotransferase (AST) prior to each infusion with ELZONRIS. Temporarily withhold ELZONRIS if the transaminases rise to greater than 5 times the upper limit of normal (ULN) and resume treatment upon normalization or when resolved

ADVERSE REACTIONS:

The most common adverse reactions in the clinical trials (incidence ≥ 30%) are capillary leak syndrome, nausea, fatigue, peripheral edema, pyrexia, and weight increase. The most common laboratory abnormalities (incidence ≥ 50%) are decreases in albumin, platelets, hemoglobin, calcium, sodium, and increases in glucose, ALT, and AST.

Please see full Prescribing Information, including Boxed WARNING.

To report SUSPECTED ADVERSE REACTIONS, contact Stemline Therapeutics, Inc. at 1-877-332-7961 or contact the FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.

IMPORTANT SAFETY INFORMATION

INDICATION

  • ELZONRIS is a CD123-directed cytotoxin for the treatment of blastic plasmacytoid dendritic cell neoplasm (BPDCN) in adults and in pediatric patients 2 years and older

IMPORTANT SAFETY INFORMATION

Boxed WARNING: CAPILLARY LEAK SYNDROME

  • Capillary Leak Syndrome (CLS), which may be life-threatening or fatal, can occur in patients receiving ELZONRIS. Monitor for signs and symptoms of CLS and take actions as recommended

WARNINGS AND PRECAUTIONS

Capillary Leak Syndrome

  • ELZONRIS can cause capillary leak syndrome (CLS), which may be life-threatening or fatal if not properly managed. The overall incidence of CLS in clinical trials was 55% in patients receiving ELZONRIS, including 46% in Grades 1 or 2, 6% in Grade 3, 1% in Grade 4, and 2 fatal events. Common signs and symptoms (incidence ≥ 20%) associated with CLS that were reported during treatment with ELZONRIS include hypoalbuminemia, edema, weight gain, and hypotension
  • Before initiating therapy with ELZONRIS, ensure that the patient has adequate cardiac function and serum albumin is ≥ 3.2 g/dL
  • During treatment with ELZONRIS, ensure that serum albumin levels are ≥ 3.5 g/dL and have not been reduced by ≥ 0.5 g/dL from the albumin value measured prior to dosing initiation of the current cycle. Monitor serum albumin levels prior to the initiation of each dose or more often as indicated clinically thereafter. Additionally, assess patients for other signs or symptoms of CLS, including weight gain, new onset or worsening edema including pulmonary edema, hypotension, or hemodynamic instability
  • Counsel patients to seek immediate medical attention should signs or symptoms of CLS occur at any time

Hypersensitivity Reactions

  • ELZONRIS can cause severe hypersensitivity reactions. Grade 3 or higher events were reported in 10% of patients in clinical trials. Monitor patients for hypersensitivity reactions during treatment with ELZONRIS. Interrupt ELZONRIS infusion and provide supportive care as needed if a hypersensitivity reaction should occur. If the reaction is severe, discontinue ELZONRIS permanently

Hepatotoxicity

  • Elevations in liver enzymes can occur with ELZONRIS. Grade 3 or higher elevations in liver enzymes occurred in approximately 40% of patients in clinical trials
  • Monitor alanine aminotransferase (ALT) and aspartate aminotransferase (AST) prior to each infusion with ELZONRIS. Temporarily withhold ELZONRIS if the transaminases rise to greater than 5 times the upper limit of normal (ULN) and resume treatment upon normalization or when resolved

ADVERSE REACTIONS:

The most common adverse reactions in the clinical trials (incidence ≥ 30%) are capillary leak syndrome, nausea, fatigue, peripheral edema, pyrexia, and weight increase. The most common laboratory abnormalities (incidence ≥ 50%) are decreases in albumin, platelets, hemoglobin, calcium, sodium, and increases in glucose, ALT, and AST.

Please see full Prescribing Information, including Boxed WARNING.

To report SUSPECTED ADVERSE REACTIONS, contact Stemline Therapeutics, Inc. at 1-877-332-7961 or contact the FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.