Future Landscape of BPDCN Management

EP: 1.Clinical Presentation and Diagnosis of BPDCN

EP: 2.Challenges and Unmet Needs in Transplant-Ineligible BPDCN Patients

EP: 3.Current Therapies and Strategies for Transplant-Ineligible BPDCN Patients

EP: 4.Improving BPDCN Recognition and Diagnosis

EP: 5.Treatment Considerations for Fit Elderly BPDCN Patients

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EP: 6.Future Landscape of BPDCN Management

Case: Management of Elderly Transplant-Ineligible BPDCN Patients

Clinical Presentation:

• A 74-year-old lady presents with fatigue, bruising like lesion on upper chest, and shortness of breath that had been present for around 6 months.

Initial Clinical Workup and Diagnosis:

• Initially suspected to have acute myeloid leukemia (AML) based on preliminary workup at a community oncology clinic.
• Referred to academic cancer center for further evaluation after not responding well to initial AML treatment.
• PMH: Hypertension, Type II Diabetes, H/O MI in 2015
• PE: Notable for pallor, petechiae, and no lymphadenopathy or hepatosplenomegaly
• ECOG PS =2
• Labs: WBC 5 x 10 K/uL, Hb 7.8 g/dL, platelets 25x 109 /L.
• Peripheral smear shows 70% blasts.
• Bone marrow biopsy showed numerous immature cells felt to represent acute leukemia.
• Cytogenetics: Complex karyotype
• LDH elevated at 1200 u/L
• Lives in assisted living facility, requires assistance with activities of daily living.
• No available matched sibling or unrelated donor identified.
• Upon referral to the academic center, pathology was reviewed, and additional material assessed leading to diagnoses of BPDCN.

Initial Treatments:

• Patient was initiated on:
  • Standard-dose cytarabine 150 mg/m2 continuous infusion x 7 days with idarubicin 12 mg/m2 or daunorubicin 60–90 mg/m2 x 3 days10
• After failure to achieve remission, she received:
  • Tagraxofusp 12 mcg/kg IV over 15 minutes once daily on days 1–5 of a 21-day cycle

This is a synopsis of a Case-Based Peer Perspectives series featuring Gary Schiller, MD, of UCLA David Geffen School of Medicine.

Gary Schiller, MD, Chief of the Hematology Stem Cell Transplant Program at David Geffen School of Medicine, UCLA, discussed the future landscape of blastic plasmacytoid dendritic cell neoplasm (BPDCN) management. Dr. Schiller mentioned that a new drug targeting the same antigen as tagraxofusp is expected to be approved for BPDCN management. This immunogen has been studied both in the initial presentation of the disease and in patients who have sustained relapse. The drug is considered effective, with a strategy similar to tagraxofusp but without the associated capillary leak syndrome. Dr. Schiller anticipates that this new drug will take a prominent position in the management of BPDCN.

For patients whose disease relapses on these treatments, which is an expected occurrence, Dr. Schiller suggested that a venetoclax-based regimen with hypomethylating agents may be tried, as there are reports of favorable outcomes with this approach.

Regarding younger patients, Dr. Schiller advised allogeneic bone marrow transplantation, considering the high risk of relapse even in patients who undergo allogeneic transplant with curative intent. He recommended assessing the spinal fluid and administering prophylactic therapy for at least two to four doses before the transplant, followed by reassessment after hematopoietic recovery and engraftment. Although this approach has not been prospectively validated, Dr. Schiller noted that it aligns with his clinical experience in managing younger patients with BPDCN.

*Video synopsis is AI-generated and reviewed by Targeted Oncology editorial staff.