Blastic plasmacytoid dendritic cell neoplasm of the uterus

Abstract

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.

Keywords: BPDCN; hematologic malignancy; aggressive