Plasmacytoid Dendritic Cell Leukemia: A Rapidly Evolving Disease Presenting with Skin Lesions Sensitive to Radiotherapy plus Hyperthermia (original) (raw)
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Dendritic Cell Leukemia: a Review
Current Oncology Reports, 2020
Purpose of Review The purpose of this review was to summarize the clinical, diagnostic, and therapeutic features of blastic plasmacytoid dendritic cell neoplasm (BPDCN). Recent Findings Several case reports and series revealed new clinical, molecular, diagnostic, and therapeutic aspects of the disease. The clinical presentation diversity has been confirmed, with frequent leukemic non-cutaneous or rare atypical manifestations. The clonal evolution in the development of BPDCN has not been sufficiently elucidated. Although certain immunophenotypic markers (CD4, TCL1, CD123, CD56, CD303) are indicative of BPDCN, the diagnosis remains in certain cases challenging. Adult (ALL)-type chemotherapy followed by hematopoietic stem cell transplantation (HSCT) is related to a favorable outcome, while chemotherapy alone seems enough in children. Future studies should continue to investigate whether CD123-directed therapies could be utilized. Summary BPDCN is a rare aggressive malignancy that needs an aggressive induction therapy. Although a diagnostic consensus is still lacking, and large retrospective studies are also needed to obtain standardized treatment guidelines, the future perspectives are encouraging, because of novel therapeutic agents that are under investigation.
Case Reports in Transplantation, 2011
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.
The American Journal of Dermatopathology, 2009
Blastic plasmacytoid dendritic cell (BPDC) neoplasm, formerly called blastic natural killer cell lymphoma or CD4+/CD56+ hematodermic neoplasm, is a rare tumor entity, now regarded to be derived from the plasmacytoid dendritic cell (PDC) lineage. Because over 90% of patients present with skin lesions usually early in their disease, dermatologists have to be familiar with the specific diagnostic features and the clinical course of this devastating disease. We present a woman with a long standing solitary skin tumor of BPDC neoplasm, who experienced a deleterious clinical course, which is typical for this disease. Phenotypic and karyotypic characteristics distinguishing this tumor from myelomonocytic leukemia with skin involvement are presented.
Plasmacytoid dendritic cell tumor: A case report
Turkish Journal of Hematology, 2011
A 62-year-old man presented with a painless eruption on his arms and trunk. Physical examination showed 2 well-demarcated erythematous plaques on the anterior trunk and 6 purple-red papules on the back and upper extremities. Blood chemistry and computed tomography results were normal. Herein we describe a patient with plasmacytoid dendritic cell neoplasm in the absence of systemic symptoms.
Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion, 2015
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a distinct and rare neoplastic entity and was classified as a subgroup of acute myeloblastic leukemia by the WHO in 2008. The median survival of patients was 15.2 months in a large case series. Allogeneic or autologous bone marrow transplantation has been recommended by some reports because of the disease's poor prognosis. We present three patients who presented with both skin and bone marrow infiltration. A 57-year-old man, a 62-year-old woman, a 64-year-old man were admitted to our outpatient clinic because of skin lesions. All of the patient's had bone marrow infiltration with positivity of the CD4, CD56, and CD123 staining. Survival of the patient's were 42, 6 and 12 months, respectively. Two of the patients who presented as blastic form didn't respond to any chemotherapy. BPDCN is a difficult disease to diagnosis and manage. CD4, CD56, CD123, CD303, and T cell leukemia/lymphoma 1. Cutaneous lesions can pres...
Novel treatment of blastic plasmacytoid dendritic cell neoplasm: A case report
Medicine, 2017
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. 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. 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. Bone marrow biopsy and radiologic work-up showed no evidence of extracutaneous involvement. The patient attained partial remission after undergoing 2 rounds of cyc...
Excessive therapeutic response in a case of blastic plasmacytoid dendritic cell neoplasm
Clinical advances in hematology & oncology : H&O, 2012
A 65-year-old African American man had been followed in the hematology-oncology clinic since 2003 for monoclonal gammopathy of unknown significance (MGUS) and anemia. Bone marrow biopsy at the time of initial presentation was unremarkable. In August 2008, he began to develop violaceous cutaneous lesions, generalized lymphadenopathy, and pancytopenia. Punch biopsy of the left arm revealed superficial and deep perivascular and interstitial infiltrates of small-to intermediate-sized cells. A bone marrow biopsy in October 2008 showed infiltration of mononuclear cells of similar morphology and immunohistochemical phenotype (positive for CD4 and CD56). Additionally, flow cytometry of a cervical lymph node biopsy from the same month demonstrated a large population of cells expressing CD4, CD56, and CD123. Based on these findings, the diagnosis of blastic plasmacytoid dendritic cell neoplasm, formerly known as CD4-positive/CD56-positive hematodermic neoplasm or blastic natural killer cell lymphoma, was made. From November 2008 to June 2009, the patient received 8 cycles of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) chemotherapy, with a good response. His cutaneous lesions and palpable lymph nodes rapidly decreased in size after the third cycle. Positron emission tomography (PET)/computed tomography (CT) scans indicated complete response after the eighth cycle. However, in August 2009, while evaluation for stem cell transplantation was being arranged, he again developed similar extensive cutaneous lesions over his