1. BPCDN is a rare hematologic neoplasm with poor prognosis.
2. It can involve cutaneous and CNS systems.
3. Recent breakthrough is the discovery of CD 123 is a targetable marker. A new drug tagraxofusp was approved in Dec 2018.
Work up should include work up for AML:
- CBC, coag profile, CMP, LDH, uric acid, peripheral smear
-bmbx
-CSF/IT chemo and flow
-NGS for myeloid mutation
- Peripheral flow --> CD 123, CD 4, CD 56
Exam: skin: violaceous nodules , bx skin lesion, mSWAT scoring
Treatment:
Ideally refer to academic center for CD 123 based clinical trials
CD 123+ chemo ( hyer CVAD) with IT chemo ( alternating methotrexate with cytarabine) followed by HSCT in CR1
In the community setting ALL like regimen HCVAD +/- Vencleta
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