Improving BPDCN Recognition and Diagnosis

Opinion
Video

Dr. Schiller will discuss the red flag symptoms that increase suspicion of BPDCN, the process for diagnosis, and any challenges with differential diagnosis. Dr. Schiller will also provide strategies and resources to improve community recognition and diagnosis of this rare disease.

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 symptom complex that should heighten one's sensitivity for making a diagnosis of blastic plasmacytoid dendritic cell neoplasm (BPDCN). Dr. Schiller emphasized that generalized ecchymosis is not common in acute myeloid leukemia (AML) unless there is associated disseminated intravascular coagulation. The typical hemorrhagic manifestation in AML is thrombocytopenic bleeding, such as petechiae and bleeding from mucosal sites.

Dr. Schiller advised that patients with an ecchymotic lesion should be carefully evaluated, as the lesion may be a cutaneous manifestation of the bone marrow disease, which would be a tip-off for BPDCN. While older age is a factor, it is not a strong indicator, as the incidence of AML also increases with age.

The constellation of cutaneous findings, pancytopenia, and bone marrow morphology with generalized replacement of the marrow by large mononuclear cells with nucleoli and the absence of cytoplasmic granules should raise suspicion for BPDCN. Dr. Schiller noted that the diagnosis relies on the pathologist running the appropriate probes, which are commercially available but may not always be utilized.

Assessment for CD123 positivity with concurrent CD34 and CD4 expression might be a subspecialty area that hematopathologists are familiar with, but general pathologists may not be. Dr. Schiller emphasized the importance of the hematologist ensuring that an adequate hematopathology assessment by flow cytometry and immunohistochemistry is performed to yield the correct diagnosis initially, as the diagnosis will not come from cytogenetics or molecular features alone.

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

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