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

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...

Full description

Saved in:
Bibliographic Details
Main Authors: Amir Steinberg, Rina Kansal, Matthew Wong, Angela Lopez, Stephen Lim, Jean Lopategui, Michael Lill
Format: Article
Language:English
Published: Wiley 2011-01-01
Series:Case Reports in Transplantation
Online Access:http://dx.doi.org/10.1155/2011/651906
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1849690053489459200
author Amir Steinberg
Rina Kansal
Matthew Wong
Angela Lopez
Stephen Lim
Jean Lopategui
Michael Lill
author_facet Amir Steinberg
Rina Kansal
Matthew Wong
Angela Lopez
Stephen Lim
Jean Lopategui
Michael Lill
author_sort Amir Steinberg
collection DOAJ
description 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.
format Article
id doaj-art-ecb27855a06c4e4b88de7194dbaa0955
institution DOAJ
issn 2090-6943
2090-6951
language English
publishDate 2011-01-01
publisher Wiley
record_format Article
series Case Reports in Transplantation
spelling doaj-art-ecb27855a06c4e4b88de7194dbaa09552025-08-20T03:21:26ZengWileyCase Reports in Transplantation2090-69432090-69512011-01-01201110.1155/2011/651906651906Good Clinical Response in a Rare Aggressive Hematopoietic Neoplasm: Plasmacytoid Dendritic Cell Leukemia with No Cutaneous Lesions Responding to 4 Donor Lymphocyte Infusions Following TransplantAmir Steinberg0Rina Kansal1Matthew Wong2Angela Lopez3Stephen Lim4Jean Lopategui5Michael Lill6Cedars-Sinai Medical Center, 8700 Beverly Boulevard, Los Angeles, CA 90048, USACedars-Sinai Medical Center, 8700 Beverly Boulevard, Los Angeles, CA 90048, USACedars-Sinai Medical Center, 8700 Beverly Boulevard, Los Angeles, CA 90048, USACedars-Sinai Medical Center, 8700 Beverly Boulevard, Los Angeles, CA 90048, USACedars-Sinai Medical Center, 8700 Beverly Boulevard, Los Angeles, CA 90048, USACedars-Sinai Medical Center, 8700 Beverly Boulevard, Los Angeles, CA 90048, USACedars-Sinai Medical Center, 8700 Beverly Boulevard, Los Angeles, CA 90048, USABlastic 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.http://dx.doi.org/10.1155/2011/651906
spellingShingle Amir Steinberg
Rina Kansal
Matthew Wong
Angela Lopez
Stephen Lim
Jean Lopategui
Michael Lill
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
Case Reports in Transplantation
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
title_full 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
title_fullStr 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
title_full_unstemmed 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
title_short 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
title_sort 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
url http://dx.doi.org/10.1155/2011/651906
work_keys_str_mv AT amirsteinberg goodclinicalresponseinarareaggressivehematopoieticneoplasmplasmacytoiddendriticcellleukemiawithnocutaneouslesionsrespondingto4donorlymphocyteinfusionsfollowingtransplant
AT rinakansal goodclinicalresponseinarareaggressivehematopoieticneoplasmplasmacytoiddendriticcellleukemiawithnocutaneouslesionsrespondingto4donorlymphocyteinfusionsfollowingtransplant
AT matthewwong goodclinicalresponseinarareaggressivehematopoieticneoplasmplasmacytoiddendriticcellleukemiawithnocutaneouslesionsrespondingto4donorlymphocyteinfusionsfollowingtransplant
AT angelalopez goodclinicalresponseinarareaggressivehematopoieticneoplasmplasmacytoiddendriticcellleukemiawithnocutaneouslesionsrespondingto4donorlymphocyteinfusionsfollowingtransplant
AT stephenlim goodclinicalresponseinarareaggressivehematopoieticneoplasmplasmacytoiddendriticcellleukemiawithnocutaneouslesionsrespondingto4donorlymphocyteinfusionsfollowingtransplant
AT jeanlopategui goodclinicalresponseinarareaggressivehematopoieticneoplasmplasmacytoiddendriticcellleukemiawithnocutaneouslesionsrespondingto4donorlymphocyteinfusionsfollowingtransplant
AT michaellill goodclinicalresponseinarareaggressivehematopoieticneoplasmplasmacytoiddendriticcellleukemiawithnocutaneouslesionsrespondingto4donorlymphocyteinfusionsfollowingtransplant