Novel treatment of blastic plasmacytoid dendritic cell neoplasm: A case report.
|Abstract||Blastic plasmacytoid dendritic cell neoplasm (BPDCN), derived from precursors of plasmacytoid dendritic cells, is a rare and aggressive malignancy with frequent cutaneous involvement. Although cutaneous lesions are often chemosensitive, BPDCN portends a poor prognosis as most patients relapse after developing drug resistance.|
Sustained complete remission of a limited-stage blastic plasmacytoid dendritic cell neoplasm followed by a simultaneous combination of low-dose DeVIC therapy and radiation therapy: a case report and review of the literature.
|Publication Year Start||2018-01-01|
PMID- 29390581 OWN - NLM STAT- MEDLINE DCOM- 20180212 LR - 20180212 IS - 1536-5964 (Electronic) IS - 0025-7974 (Linking) VI - 96 IP - 51 DP - 2017 Dec TI - Novel treatment of blastic plasmacytoid dendritic cell neoplasm: A case report. PG - e9452 LID - 10.1097/MD.0000000000009452 [doi] AB - RATIONALE: Blastic plasmacytoid dendritic cell neoplasm (BPDCN), derived from precursors of plasmacytoid dendritic cells, is a rare and aggressive malignancy with frequent cutaneous involvement. Although cutaneous lesions are often chemosensitive, BPDCN portends a poor prognosis as most patients relapse after developing drug resistance. PATIENT CONCERNS: We report a case of a 65-year-old man who presented with a rapidly enlarging hyperpigmented plaque on his shoulder with subsequent similarly appearing macules and plaques on his chest, back, and neck. DIAGNOSIS: Skin biopsy revealed a dense adnexocentric dermal infiltrate of immature blastoid cells without epidermal involvement. The infiltrate was immunoreactive for CD4, CD56, CD123, and Bcl-2, but negative for CD3, CD8, CD30, MPO, EBER, and ISH. The patient was diagnosed with BPDCN based on these cell markers. INTERVENTION: Bone marrow biopsy and radiologic work-up showed no evidence of extracutaneous involvement. The patient attained partial remission after undergoing 2 rounds of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP regimen) before autologous stem cell transplantation, however, he quickly relapsed and developed new cutaneous lesions. OUTCOMES: The patient was treated with venetoclax, a Bcl-2 inhibitor, and exhibits complete resolution of prior skin findings and continues to remain free of new cutaneous lesions 10 months posttreatment initiation with venetoclax. LESSONS: Herein, we present a case that supports the use of venetoclax, a Bcl-2 inhibitor, in the off-label treatment of BPDCN with Bcl-2 overexpression. Only 1 prior case has reported the off-label use of venetoclax for the treatment of BPDCN. This case highlights a novel therapeutic option for BPDCN patients unresponsive to traditional treatment. CI - Copyright (c) 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved. FAU - Grushchak, Solomiya AU - Grushchak S FAU - Joy, Cossette AU - Joy C FAU - Gray, Arielle AU - Gray A FAU - Opel, Dan AU - Opel D FAU - Speiser, Jodi AU - Speiser J FAU - Reserva, Jeave AU - Reserva J FAU - Tung, Rebecca AU - Tung R FAU - Smith, Scott E AU - Smith SE LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R RN - 0 (CD4 Antigens) RN - 0 (CD56 Antigen) SB - AIM SB - IM MH - Aged MH - CD4 Antigens/metabolism MH - CD56 Antigen/metabolism MH - Dendritic Cells/*pathology MH - Humans MH - Male MH - Skin/cytology/pathology MH - Skin Neoplasms/*diagnosis/pathology PMC - PMC5758283 EDAT- 2018/02/03 06:00 MHDA- 2018/02/13 06:00 CRDT- 2018/02/03 06:00 PHST- 2018/02/03 06:00 [entrez] PHST- 2018/02/03 06:00 [pubmed] PHST- 2018/02/13 06:00 [medline] AID - 10.1097/MD.0000000000009452 [doi] AID - 00005792-201712220-00168 [pii] PST - ppublish SO - Medicine (Baltimore). 2017 Dec;96(51):e9452. doi: 10.1097/MD.0000000000009452.