Overview of BPDCN

What Is BPDCN?

BPDCN is a deadly hematologic cancer.1

BPDCN = Blastic Plasmacytoid Dendritic Cell Neoplasm
BPDCN = Blastic Plasmacytoid Dendritic Cell Neoplasm

Evolving nomenclature

BPDCN emerges as a unique myeloid neoplasm after a long history of reclassification.2 Previous names for BPDCN included blastic NK-cell lymphoma and agranular CD4+/CD56+ hematodermic neoplasm.3,4 The World Health Organization (WHO) officially named this malignancy BPDCN in 2008 and subsequently classified it as a separate myeloid neoplasm in 2016.2,5

HISTORY OF WORLD HEALTH ORGANIZATION CLASSIFICATION FOR BPDCN

2001

Blastic NK-cell lymphoma3

2005

Agranular CD4+ CD56+ hematodermic neoplasm4

2008

BPDCN as a subset of AML5

2016

BPDCN as a unique myeloid neoplasm2

Patient population

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

PATIENT POPULATION1,6

Patient Population With BPDCN
Patient Population With BPDCN

Have you seen a case of BPDCN?

Similarity to other hematologic malignancies has contributed to misdiagnosis of BPDCN.1,7‐9

BPDCN MAY BE MISTAKEN FOR1,7-9

BPDCN similarity to other hematologic malignancies
BPDCN similarity to other hematologic malignancies

NK = natural killer; AML = acute myeloid leukemia; NHL = non-Hodgkin’s lymphoma; ALL = acute lymphoblastic leukemia; MDS = myelodysplastic syndrome; CMML = chronic myelomonocytic leukemia.

References: 1 Pagano L, Valentini CG, Grammatico S, Pulsoni A. Blastic plasmacytoid dendritic cell neoplasm: diagnostic criteria and therapeutical approaches. Br J Haematol. 2016;174(2):188-202. 2. Chan JKC, Jaffe ES, Ralfkiaer E. Blastic NK-cell lymphoma. In: Jaffe ES, Harris NL, Stein H, Vardiman JW, eds. WHO Classification. Tumors of Haematopoietic and Lymphoid Tissues. Lyon, France: IARC Press; 2001:214-215. 3. Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas. Blood. 2005;105(10):3768-3785. 4. Facchetti F, Jones DM, Petrella T. Blastic plasmacytoid dendritic cell neoplasm. In: Swerdlow SH, Campo E, Harris NL, et al, eds. WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues. 4th ed. Lyon, France: IARC Press; 2008:145-147. 5. Arber DA, Orazi A, Hasserjian R, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016;127(20):2391-2405. 6. Pagano L, Valentini CG, Pulsoni A, et al. Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study. Haematologica. 2013;98(2):239-246. 7. Laribi K, Denizon N, Besançon A, et al. Blastic plasmacytoid dendritic cell neoplasm: from origin of the cell to targeted therapies. Biol Blood Marrow Transplant. 2016;22(8):1357-1367. 8. Riaz W, Zhang L, Horna P, Sokol L. Blastic plasmacytoid dendritic cell neoplasm: update on molecular biology, diagnosis, and therapy. Cancer Control. 2014;21(4):279-289. 9. León-Martinez G, Meillón-García L, Morales-Polanco M, Soler-Montecinos L, Ortiz-Hidalgo C. Unusual morphologic presentations of blastic plasmacytoid dendritic cell neoplasm: report of two cases misdiagnosed as melanoma and leprosy. Int J Surg Pathol. 2014;22(1):76-82.

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.