Venetoclax monotherapy for cutaneous blastic plasmacytoid dendritic cell neoplasm
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LETTER TO THE EDITOR
Venetoclax monotherapy for cutaneous blastic plasmacytoid dendritic cell neoplasm Matthew Schwede 1 Gabriel N. Mannis 1
&
Irena T. Tan 1 & Danielle F. Atibalentja 1 & Meghan M. Dickman 2 & Kerri E. Rieger 2,3 &
Received: 2 September 2020 / Accepted: 17 September 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Dear Editor, Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy arising from precursors of plasmacytoid dendritic cells, with immunohistochemical staining characteristically positive for CD4, CD56, CD123, and TCL-1 [1, 2]. BPDCN most commonly presents with cutaneous lesions [3], though many patients also have lymphadenopathy, splenomegaly, and cytopenias [4]. BPDCN may be rapidly fatal without treatment, and although multi-agent chemotherapy has historically yielded complete remissions in select patients, these remissions are typically transient [4], and BPDCN is thought to be incurable without allogeneic hematopoietic cell transplant [5]. Tagraxofusp, a CD123-directed cytotoxin, was recently FDA-approved for the treatment of BPDCN based on complete and overall response rates of 72% and 90%, respectively, in newly diagnosed patients [6]. However, tagraxofusp is associated with significant toxicities, including a potentially fatal capillary leak syndrome [6]. Recently, in vitro studies showed that BPDCN is dependent on B-cell lymphoma 2 (BCL-2) protein to prevent apoptosis, and in vivo experiments demonstrated improved survival of animals treated with the oral BCL-2 inhibitor, venetoclax [7]. Accordingly, a handful of recent reports describe using venetoclax—either alone or in combination—for both newly diagnosed and relapsed/ refractory BPDCN [7–9].
* Matthew Schwede [email protected] 1
Division of Hematology, Department of Medicine, Stanford University School of Medicine/Stanford Cancer Institute, 875 Blake Wilbur Drive, Palo Alto, Stanford, CA 94304, USA
2
Department of Dermatology, Stanford University School of Medicine, Stanford, CA, USA
3
Department of Pathology, Stanford University School of Medicine, Stanford, CA, USA
Herein, we report the case of an 88-year-old man who presented to his dermatologist with a ten month history of progressive, non-ulcerated, violaceous cutaneous plaques. These initially appeared on his right lower leg with subsequent involvement of the left leg, chest, scalp, and back. The plaques were non-pruritic and non-painful, and he reported no improvement with topical steroids. Punch biopsies revealed a dense, nodular and sheet-like, monomorphous infiltrate involving the dermis and extending into the subcutis. Tumor cells were medium in size with blastoid appearance (Fig. 1a, b). Immunohistochemical studies showed the cells were positive for CD4, CD56, CD123, TCL-1, and BCL-2, consistent with BPDCN, and bone marrow biopsy showed no evidence of involvement. PET/CT showed circumferential skin thickening and hypermetabolism in the right lower extremity, with hypermetabolic inguinal and likely femo
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