Future Landscape of BPDCN Management

Opinion
Video

Dr. McCloskey discusses the limitations of current treatment options for BPDCN, as well as the emerging research and data that are most anticipated in the field.

Clinical Presentation:

A 67-year-old man referred from dermatologist.

Referred initially to dermatologist by PCP for progressive, persistent, cutaneous nodules that patient first noticed 3 weeks prior

Initial Clinical Workup and Diagnosis:

ROS: Fatigue, 5 kg weight loss over 3 moths

PMH: Sinusitis; no major comorbidities

PE: Notable for multiple purpuric nodules (measuring up to 5 cm on arms, legs, torso). No palpable adenopathy, hepatosplenomegaly

ECOG PS =1

Labs: WBC 14.1 x 103/uL, Hb 8.9 g/dL, platelets 54 x 103/uL. Differential revealed 12% blasts, 32% neutrophils, 16% monocytes, 40% lymphocytes.

Skin: purpuric nodule

Peripheral blood smear: blastic cells with large and round or slightly irregular nuclei; blast cytoplasm stained greyish blue without granules or Auer rods

Bone marrow biopsy showed 40% blasts by morphology; 80% cellular marrow with interstitial infiltrate.

IHC of neoplastic cells: CD123, CD4, CD56, TCL1 positive

Flow cytometry:

CD4, CD56, CD123 were positive;

CD34 and T- and B-cell lineage-specific markers were negative

Cytogenetics: 46 XY

Lumbar puncture did not indicate CNS involvement

The patient was ultimately diagnosed with BPDCN based on clinical and histopathological findings.

Initial Treatments:

Tagraxofusp initiated:

Initial dose 12 mcg/kg as per package label for frontline therapy and achieves CR after 1 cycle of therapy.

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