Overview of BPDCN

BPDCN Presentation

BPDCN often presents in the skin and bone marrow.1-5 Due to the variable dermatologic and hematologic presentation of BPDCN, accurate diagnosis is critical.6

Use the tabs below to explore features that a patient with BPDCN may exhibit.

Dermatologic
Hematologic

~85% to 90% of patients with BPDCN present with skin lesions1-3

Nodular Lesions in Patients With BPDCN Nodular Lesions in Patients With BPDCN
Diffuse Bruise-like Macules in Patients With BPDCN Diffuse Bruise-like Macules in Patients With BPDCN
View more skin lesions in patients with BPDCN
Bruise-Like Infiltrate on the Chest of a Patient With BPDCN
Bruise-like infiltrate presenting on the chest Reprinted by Permission of SAGE Publications, Inc.
Bruise-Like Infiltrate on the Chest of a Patient With BPDCN
Lesions on the Chest of a Patient With BPDCN Lesions on the Back of a Patient With BPDCN
Hyperpigmented presentations on the chest and back Republished with permission of Blood.
Lesions on the Chest of a Patient With BPDCN
Lesions on the Back of a Patient With BPDCN
Bruise-Like Lesion on the Face of a Patient With BPDCN Erythematous Lesions on the Trunk of a Patient With BPDCN
Bruise‐like or erythematous papules, plaques, or tumors presenting on the face and trunk Courtesy of Shapiro R, et al. J Cell Sci Ther. S8:008. Doi:10.4172/2157-7013.S8-008.8.
Bruise-Like Lesion on the Face of a Patient With BPDCN
Erythematous Lesions on the Trunk of a Patient With BPDCN

~60% to 90% of patients present with leukemic disease4,5

If untreated, BPDCN rapidly progresses to an aggressive leukemic disease.4,7 The median overall survival for BPDCN is approximately 8 to 14 months after diagnosis.7,8

Primary sites of involvement include4,9

  • Peripheral blood
  • Bone marrow

Secondary sites of involvement include4,10

  • Lymph nodes
  • Viscera

BIOPSIES OF SKIN, BONE MARROW, OR SECONDARY SITES ARE CRITICAL TO DIAGNOSIS.6

References: 1. Sullivan JM, Rizzieri DA. Treatment of blastic plasmacytoid dendritic cell neoplasm. Hematology Am Soc Hematol Educ Program. 2016;2016(1):16-23. 2. 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. 3. Julia F, Petrella T, Beylot-Barry M, et al. Blastic plasmacytoid dendritic cell neoplasm: clinical features in 90 patients. Br J Dermatol. 2013;169(3):579-586. 4. 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. 5. Herling M, Jones D. CD4+/CD56+ hematodermic tumor: the features of an evolving entity and its relationship to dendritic cells.Am J Clin Pathol. 2007;127(5):687-700. 6. Fachetti F, Cigognetti M, Fisogni S, Rossi G, Lonardi S, Vermi W. Neoplasms derived from plasmacytoid dendritic cells. Mod Pathol. 2016;29(2):98-111. 7. 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. 8. 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. 9. Reichard KK. Blastic plasmacytoid dendritic cell neoplasm: how do you distinguish it from acute myeloid leukemia? Surg Pathol Clin. 2013;6(4):743-765. 10. 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.

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.