title: Good clinical response in a rare aggressive hematopoietic neoplasm: plasmacytoid dendritic cell leukemia with no cutaneous lesions responding to 4 donor lymphocyte infusions following transplant.
abstract: Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) is a rare and aggressive malignancy that usually presents with diffuse cutaneous lesions. While a favorable response to therapy occurs in a majority of cases, a sustained long-term response is uncommon. Most patients subsequently relapse within a year. In the following report, we present the case of a 41-year-old woman who has not displayed many of the clinical features traditionally associated with BPDCN. The patient received sporadic chemotherapy treatment over the course of 2 years, before undergoing an allogeneic stem cell transplant. Although she ultimately relapsed following her transplant, her disease has repeatedly returned into remission after donor lymphocyte infusion (DLI). Currently, the patient is in remission following her fourth DLI. We believe that allogeneic transplantation should be considered as front-line therapy for the treatment of this rare malignancy.
authors.affiliation: Cedars-Sinai Medical Center, 8700 Beverly Boulevard, Los Angeles, CA 90048, USA ; Division of Hematology-Oncology, Mount Sinai Medical Center, 1425 Madison Avenue, New York City, NY 10029, USA.
authors.forename: Amir
authors.initials: A
authors.lastname: Steinberg
authors.forename: Rina
authors.initials: R
authors.lastname: Kansal
authors.forename: Matthew
authors.initials: M
authors.lastname: Wong
authors.forename: Angela
authors.initials: A
authors.lastname: Lopez
authors.forename: Stephen
authors.initials: S
authors.lastname: Lim
authors.forename: Jean
authors.initials: J
authors.lastname: Lopategui
authors.forename: Michael
authors.initials: M
authors.lastname: Lill