Clinical Presentation and Diagnosis of BPDCN

Opinion
Video

Dr. Schiller will discuss the key clinical and pathologic features that distinguish BPDCN from other malignancies, as well as the potential differential diagnoses that share a similar immunophenotype.

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, presented a case of a 74-year-old patient with a history of fatigue, a bruising leg lesion on the upper chest, and shortness of breath that had been present for six months. The patient's medical history included hypertension, type 2 diabetes, and a myocardial infarction. Dr. Schiller noted that the patient was initially suspected to have acute myeloid leukemia (AML) at a community oncology clinic and was later referred to an academic medical center when she did not respond to her initial therapy.

According to Dr. Schiller, the patient's physical exam was notable for pallor, ecchymosis, and the bruised lesion on the upper chest. Laboratory findings showed anemia, thrombocytopenia, and a large number of blasts in the peripheral blood. A bone marrow biopsy revealed numerous immature cells, initially thought to represent acute leukemia. The patient had a high burden of disease with a high lactate dehydrogenase (LDH) level and underwent initial therapy with cytarabine and daunorubicin, which Dr. Schiller questioned given her age.

After the disease failed to respond to conventional induction therapy, the patient was referred to an academic medical center, where the pathology was reviewed, and a diagnosis of blastic plasmacytoid dendritic cell neoplasm (BPDCN) was made. Dr. Schiller emphasized the importance of the pathological presentation and diagnostic findings in this case. He noted that the patient's bruised lesion, which failed to resolve, was a clinical indication of a cutaneous manifestation of the bone marrow disease, which is common in BPDCN.

Dr. Schiller explained that a detailed immunohistochemical evaluation of the bone marrow would identify a distinct pattern of antigen expression on the neoplastic cells, with positivity for CD4, CD56, CD123, and TCL1. Flow cytometry would also show positivity for CD4, CD123, CD56, and CD34, with an absence of T- and B-lymphoid lineage-specific markers. This unique antigen pattern is characteristic of BPDCN, a distinct entity that may present with high-risk cytogenetics or normal cytogenetics.

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

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