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.

Haematopoietic cell transplantation for blastic plasmacytoid dendritic cell neoplasm: a North American multicentre collaborative study

“…One plausible explanation is that in our series a higher proportion of patients were transplanted in CR1 compared to the EBMT series (76% vs. 56%), suggesting a limited role for myeloablative regimens in this setting. Anecdotal data supports the role of donor lymphocyte infusions when BPDCN relapses after an allo‐HCT (Steinberg et al , ). Consistent with the EBMT findings (Roos‐Weil et al , ), age, cell source and chronic GVHD did not affect OS in our series.…”

Blastic plasmacytoid dendritic cell neoplasm: challenges and future prospects

“…A single case report described a reasonable remission (1 year) and tolerability of oral etoposide in an older patient with relapsed BPDCN and another described partial disease control of cutaneous lesions with weekly infusions of prala-trexate 48,49. A case series supporting the use of azacytidine has also been reported as has the use of repeat courses of donor lymphocyte infusions after allogeneic HSCT relapse 50,51…”

The beneficial role of allogenic hematopoietic cell transplantation in blastic plasmacytoid dendritic cell neoplasm

“…The disease was described in 1994, and in 2008 it was included in the World Health Organization (WHO) classification for and has the tendency to disseminate via peripheral blood. Due to accompanying cytopenias, the course of the disease is similar to that of leukemia [21]. On the contrary, immunophenotypically mature cells infiltrate the extramedullary organs and the disease may resemble aggressive lymphomas.…”