The first case of blastic plasmacytoid dendritic cell neoplasm with MLL-ENL rearrangement

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a clinically aggressive hematologic malignancy arising from precursors of plasmacytoid dendritic cells that represent less than 1% of hematological malignancies. BPDCN initially presents with cutaneous involvement and a characteristic immunophenotype of CD4, CD56, and CD123 co-expression. Upon disease progression, BPDCN shows a strong predilection for bone marrow, peripheral blood, and lymph nodes, whereas manifestations in visceral organs are rare. Significant heterogeneity in clinical presentation and immunophenotypic profile makes BPDCN challenging to diagnose without an integrated approach based on patient history, clinical features, tumor pathology, and comprehensive immunohistochemical studies. Herein we report the first case of relapsed BPDCN manifesting as a unilateral testicular tumor.

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Blastic Plasmacytoid Dendritic Cell Neoplasm: First Case Report From Rwanda and Review of the Literature

Journal of Global Oncology ◽

10.1200/jgo.19.00123 ◽

2019 ◽

pp. 1-6

Author(s):

Deogratias Ruhangaza ◽

Marcellin C. Mugabe ◽

Catherine N. Kigonya ◽

Andrew A. Lane ◽

Elizabeth A. Morgan

Keyword(s):

Case Report ◽

Dendritic Cell ◽

Plasmacytoid Dendritic Cell ◽

Review Of The Literature ◽

First Case ◽

Cell Neoplasm

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Blastic plasmacytoid dendritic cell neoplasm of the uterus

Srpski arhiv za celokupno lekarstvo ◽

10.2298/sarh191111027d ◽

2020 ◽

Vol 148 (9-10) ◽

pp. 602-605

Author(s):

Predrag Djurdjevic ◽

Zeljko Todorovic ◽

Danijela Jovanovic ◽

Ivan Cekerevac ◽

Ljiljana Novkovic ◽

...

Keyword(s):

Dendritic Cell ◽

Plasmacytoid Dendritic Cell ◽

Treatment Options ◽

Bone Marrow Aspiration ◽

Skin Lesions ◽

Cervix Uteri ◽

Chromosome 2 ◽

First Case ◽

Cell Neoplasm ◽

Complete Blood Counts

Introduction. Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and very aggressive hematological malignancy derived from precursor of the plasmacytoid dendritic cell. We present a case with cervix uteri involvement without skin lesions, which is, to the best of our knowledge, the first case of BPDCN localized in the cervix. Case outline. A 66-year-old previously healthy women initially presented with a four-week history of vaginal bleeding. Gynecologic examination revealed a tumorous bleeding formation on cervix uteri. Except paleness of the skin, physical examination results were normal. Complete blood counts showed anemia and thrombocytopenia. Computed tomography scans showed an expansive tumorous formation at the level of the isthmus and cervix uteri, 60 ? 42 mm in size. Cervical biopsy was done and final pathohistological diagnosis was BPDCN. Karyotype analysis results from the bone marrow aspiration specimen demonstrated tetrasomy of chromosome 2 and monosomy of chromosome 16. The patient did not accept treatment and died two months after the initial diagnosis was established. Conclusion. Attributes such as aggressive clinical course of BPDCN, demonstrated unusual localization, infrequency, and the absence of consensus about standard treatment options, demand constructive clinical reasoning and tight cooperation between medical professionals of various fields.

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