A Case of Cutaneous Blastic Plasmacytoid Dendritic Cell Neoplasm

Xue, Ruzeng; Wu, Tieqiang; Pan, Huiqing; Gu, Yuan; Yang, Bin; Chen, Yongfeng; Jushi, Qiu

doi:10.2340/00015555-0922

Cited by 4 publications

(3 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The first manifestation of the disease is often cutaneous involvement. The majority of patients present skin involvement, while a number of cases with only skin involvement have been reported ( 8 ). The present study presents a rare case of leukemic manifestation with lack of skin involvement.…”

Section: Discussionmentioning

confidence: 99%

Blastic plasmacytoid dendritic cell neoplasm with leukemic manifestation and ETV6 gene rearrangement: A case report

Gao

Wang

Sun

et al. 2015

Experimental and Therapeutic Medicine

View full text Add to dashboard Cite

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare malignant tumor of the hemopoietic system that arises from plasmacytoid dendritic cell precursors with a highly aggressive course. BPDCN frequently involves the skin, lymph nodes, peripheral blood and bone marrow. BPDCN is known to develop leukemic dissemination as a feature of myelomonocytic leukemia in the late phase of the disease, which leads to a poorer prognosis. In the present study, a case of BPDCN with leukemic manifestation without cutaneous involvement was reported. In addition, ETS variant gene 6 (ETV6) gene rearrangement was detected in the patient. The patient relapsed soon after complete remisson and had no response to further treatment. To the best of our knowledge, this is the first reported case of BPDCN with ETV6 rearrangement. Following chemotherapy treatment, the patient suffered from severe headache in the complete remission stage; however, brain CT scans showed no significant abnormalities. Several lumbar punctures and intrathecal chemotherapy were performed, and the patient recovered gradually. Therefore, the patient was considered to suffer from central nervous system leukemia. In conclusion, implementation of lumbar punctures and preventive intrathecal chemotherapy are required in BPDCN patients with leukemic manifestation during the remission stage.

show abstract

Section: Discussionmentioning

confidence: 99%

Blastic plasmacytoid dendritic cell neoplasm with leukemic manifestation and ETV6 gene rearrangement: A case report

Gao

Wang

Sun

et al. 2015

Experimental and Therapeutic Medicine

View full text Add to dashboard Cite

show abstract

“…It was formally known as blastic natural killer (NK) lymphoma, or agranular CD4+CD56+ hematodermic neoplasm, and accounts for about 0.7% of all skin and primary malignant lymphoma [7]. The etiology of BPDCN is still unclear.…”

Section: Discussionmentioning

confidence: 99%

“…Its typical pathologic characteristics are uniformly distributed, dense, medium-sized lymphocytes in the dermis layer. Tumor cells are similar in appearance, with a medium-sized, round, or irregular nucleus, and the nucleolus is not obvious [6, 7]. Typical immunohistochemistry results include positive for CD4, CD56, negative for CD3, CD20, myeloperoxidase, and CD33.…”

Section: Discussionmentioning

confidence: 99%

Blastic Plasmacytoid Dendritic Cell Neoplasm on the Scalp

2011

View full text Add to dashboard Cite

We describe a 75-year-old Japanese woman with blastic plasmacytoid dendritic cell neoplasm. On her first visit, there were multiple areas of disseminated, elastic hard nodules with multicentric purpura on her scalp. Histopathologically, there were atypical large lymphocytes densely infiltrated through the upper dermis to subcutaneous tissue. Immunohistochemical staining revealed that these infiltrated lymphocytes were CD4, CD56 and vimentin positive. Although we administered several courses of intensive chemotherapy soon after the diagnosis, the patient died 5 months after the first visit.

show abstract

Ulcerating skin lesions from blastic plasmacytoid dendritic cell neoplasm responding to low-dose radiotherapy—a case report and literature review

Hoffmann,

Böke,

De-Colle

et al. 2024

Strahlenther Onkol

View full text Add to dashboard Cite

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy that can manifest with skin nodules and erythematous plaques. In most cases BPDCN progresses rapidly, causing multiple skin lesions and also affecting internal organs and bone marrow, warranting initiation of systemic therapies or hematopoietic stem cell transplantation (HCT). Although not curative, radiotherapy for isolated lesions might be indicated in case of (imminent) ulceration and large or symptomatic lesions. To this end, doses of 27.0–51.0 Gy have been reported. Here, we present the case of an 80-year-old male with BPDCN with multiple large, nodular, and ulcerating lesions of the thorax, abdomen, and face. Low-dose radiotherapy of 2 × 4.0 Gy was administered to several lesions, which resolved completely within 1 week with only light residual hyperpigmentation of the skin in affected areas and reliably prevented further ulceration. Radiotoxicity was not reported. Therefore, low-dose radiotherapy can be an effective and low-key treatment in selected cases of BPDCN, especially in a palliative setting, with a favorable toxicity profile.

show abstract

A Case of Cutaneous Blastic Plasmacytoid Dendritic Cell Neoplasm

Cited by 4 publications

References 10 publications

Blastic plasmacytoid dendritic cell neoplasm with leukemic manifestation and ETV6 gene rearrangement: A case report

Blastic plasmacytoid dendritic cell neoplasm with leukemic manifestation and ETV6 gene rearrangement: A case report

Blastic Plasmacytoid Dendritic Cell Neoplasm on the Scalp

Ulcerating skin lesions from blastic plasmacytoid dendritic cell neoplasm responding to low-dose radiotherapy—a case report and literature review

Contact Info

Product

Resources

About