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

Gao,Na; Wang,Xue‑Xia; Sun,Jian‑Rong; Yu,Wen‑Zheng; Guo,Nong‑Jian

doi:10.3892/etm.2015.2236

April-2015 Volume 9 Issue 4

Full Size Image

Journals

International Journal of Molecular Medicine

International Journal of Molecular Medicine is an international journal devoted to molecular mechanisms of human disease.

International Journal of Oncology

International Journal of Oncology is an international journal devoted to oncology research and cancer treatment.

Molecular Medicine Reports

Covers molecular medicine topics such as pharmacology, pathology, genetics, neuroscience, infectious diseases, molecular cardiology, and molecular surgery.

Oncology Reports

Oncology Reports is an international journal devoted to fundamental and applied research in Oncology.

Experimental and Therapeutic Medicine

Experimental and Therapeutic Medicine is an international journal devoted to laboratory and clinical medicine.

Oncology Letters

Oncology Letters is an international journal devoted to Experimental and Clinical Oncology.

Biomedical Reports

Explores a wide range of biological and medical fields, including pharmacology, genetics, microbiology, neuroscience, and molecular cardiology.

Molecular and Clinical Oncology

International journal addressing all aspects of oncology research, from tumorigenesis and oncogenes to chemotherapy and metastasis.

World Academy of Sciences Journal

Multidisciplinary open-access journal spanning biochemistry, genetics, neuroscience, environmental health, and synthetic biology.

International Journal of Functional Nutrition

Open-access journal combining biochemistry, pharmacology, immunology, and genetics to advance health through functional nutrition.

International Journal of Epigenetics

Publishes open-access research on using epigenetics to advance understanding and treatment of human disease.

Medicine International

An International Open Access Journal Devoted to General Medicine.

April-2015 Volume 9 Issue 4

Full Size Image

Case Report Open Access

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

Authors:
- Na Gao
- Xue‑Xia Wang
- Jian‑Rong Sun
- Wen‑Zheng Yu
- Nong‑Jian Guo
View Affiliations / Copyright

Affiliations: Department of Hematology, Binzhou Medical University Hospital, Binzhou, Shandong 256603, P.R. China, Department of Hematology, Central Hospital of Jinan, Shandong University School of Medicine, Jinan, Shandong 250013, P.R. China

Copyright: © Gao et al. This is an open access article distributed under the terms of Creative Commons Attribution License.
Pages: 1109-1112
|
Published online on: January 29, 2015

https://doi.org/10.3892/etm.2015.2236
Expand metrics +

Abstract

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.

View Figures

View References

1	Facchetti F, Jones DM and Petrella T: Blastic plasmacytoid dendritic cell neoplasm. Swerdlow SH, Campo E, Harris NL, et al: WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues. 4th edition. IARC Press; Lyon: pp. 145–147. 2008
2	Petrella T, Comeau MR, Maynadié M, Couillault G, et al: ‘Agranular CD4+ CD56+ hematodermic neoplasm’ (blastic NK-cell lymphoma) originates from a population of CD56+ precursor cells related to plasmacytoid monocytes. Am J Surg Pathol. 26:852–862. 2002. View Article : Google Scholar : PubMed/NCBI
3	Maki K, Arai H, Waga K, Sasaki K, Nakamura F, Imai Y, et al: Leukemia-related transcription factor TEL is negatively regulated through extracellular signal-regulated kinase-induced phosphorylation. Mol Cell Biol. 24:3227–3237. 2004. View Article : Google Scholar : PubMed/NCBI
4	Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, Ralfkiaer E, Chimenti S, Diaz-Perez JL, Duncan LM, et al: WHO-EORTC classification for cutanous lymphomas. Blood. 105:3768–3785. 2005. View Article : Google Scholar : PubMed/NCBI
5	Adachi M, Maeda K, Takekawa M, Hinoda Y, Imai K, Sugiyama S and Yachi A: High expression of CD56 (N-CAM) in a patient with cutaneous CD4-positive lymphoma. Am J Hematol. 47:278–282. 1994. View Article : Google Scholar : PubMed/NCBI
6	Chinese Society of Hematology, Chinese Medical Association and Society of Hematological Malignancies Chinese Anti-Cancer Association. A Chinese expert panel consensus on diagnosis and treatment of adult acute lymphoblastic leukemia. Zhonghua Xue Ye Xue Za Zhi. 33:789–792. 2012.(In Chinese).
7	Jin J; Chinese Society of Hematology and Chinese Medical Association. Chinese guidelines for the diagnosis and treatment of relapsed and refractory acute myelogeneous leukemia. Zhonghua Xue Ye Xue Za Zhi. 32:887–888. 2011.(In Chinese).
8	Xue R, Wu T, Pan H, Gu Y, Yang B, Chen Y and Qiu J: A case of cutaneous blastic plasmacytoid dendritic cell neoplasm. Acta Derm Venereol. 90:645–646. 2010. View Article : Google Scholar : PubMed/NCBI
9	Facchetti F, Ungari M, Marocolo D, Lonardi S and Vermi W: Blastic plasmacytoid dendritic cell neoplasm. Haematol Meet Rep. 3:1–3. 2009.
10	Lopez RG, Carron C, Oury C, Gardellin P, Bernard O and Ghysdael J: TEL is a sequence-specific transcriptional repressor. J Biol Chem. 274:30132–30138. 1999. View Article : Google Scholar : PubMed/NCBI
11	Takahashi W, Sasaki K, Kvomatsu N and Mitani K: TEL/ETV6 accelerates erythroid differentiation and inhibits megakaryocytic maturation in a human leukemia cell line UT-7/GM. Cancer Sci. 96:340–348. 2005. View Article : Google Scholar : PubMed/NCBI
12	Leroux D, Mugneret F, Callanan M, Radford-Weiss I, Dastugue N, Feuillard J, Le Mée F, Plessis G, Talmant P, Gachard N, et al: CD4(+), CD56(+) DC2 acute leukemia is characterized by recurrent clonal chromosomal changes affecting 6 major targets: a study of 21 cases by the Groupe Français de Cytogénétique Hématologique. Blood. 99:4154–4159. 2002. View Article : Google Scholar : PubMed/NCBI
13	Piccaluga PP, Paolini S, Sapienza MR and Pileri SA: Blastic plasmacytoid dendritic cell neoplasm: is it time to redefine the standard of care? Expert Rev Hematol. 5:353–355. 2012. View Article : Google Scholar : PubMed/NCBI

Journals

International Journal of Molecular Medicine

International Journal of Oncology

Molecular Medicine Reports

Oncology Reports

Experimental and Therapeutic Medicine

Oncology Letters

Biomedical Reports

Molecular and Clinical Oncology

World Academy of Sciences Journal

International Journal of Functional Nutrition

International Journal of Epigenetics

Medicine International

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

This article is mentioned in:

Abstract

Figure 1

Figure 2

Figure 3

Figure 4

Figure 5

Related Articles