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Case Reports
. 2019 Feb;98(6):e14344.
doi: 10.1097/MD.0000000000014344.

Blastic plasmacytoid dendritic cell neoplasm with unusual extracutaneous manifestation: Two case reports and literature review

Seung Eun Lee  1 , Ha Young Park  2 , Dohee Kwon  3 , Yoon Kyung Jeon  3   4 , Wook Youn Kim  5
Affiliations
Case Reports

Blastic plasmacytoid dendritic cell neoplasm with unusual extracutaneous manifestation: Two case reports and literature review

Seung Eun Lee et al. Medicine (Baltimore). 2019 Feb.

Abstract

Rationale: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy. This disease almost always presents with cutaneous involvement.

Patient concerns: The 1st patient was a 16-year-old girl who presented with recurrent epistaxis. The 2nd patient was a 17-year-old female who presented with nasal obstruction and voice change for a month.

Diagnoses: In the 1st patient, sinonasal computed tomography (CT) revealed a 2.9-cm sized, polypoid mass in the nasal cavity. In the 2nd patient, CT scans revealed a large enhancing nasopharyngeal mass involving adenoid and several small indeterminate lymph nodes at the neck. Cutaneous examination was unremarkable for either patient. Biopsy of these 2 masses and bone marrow biopsy were performed. Histologic diagnosis of the 2 cases was BPDCN.

Interventions: Both patients were treated with induction chemotherapy and received allogenic peripheral blood stem-cell transplant.

Outcomes: No relapse was observed in the 2 patients for 14 and 11 months, respectively, after transplantation. Interestingly, they had no skin lesions at initial diagnosis or during the course of their illness.

Lessons: We 1st identified nasal cavity as an unusual site of BPDCN. BPDCN should be considered in differential diagnosis of blastic leukemia with an undifferentiated and ambiguous immunophenotype despite the absence of skin lesions.

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Figures

Figure 1
Figure 1
Histologic and immunohistochemical findings of blastic plasmacytoid dendritic cell neoplasm (BPDCN) of the 1st case. (A) At low magnification, microscopic examination reveals that the nasal mucosa is diffusely expanded and infiltrated by atypical lymphoid infiltrates. (B) Infiltrative tumor cells are diffuse, monomorphic medium-sized cells with fine chromatin, irregular nuclei, and scanty cytoplasm, reminiscent of blasts. Immunohistochemically, these tumor cells show immunoreactivity for CD4 (C), CD56 (D), CD123 (E), and focal TCL1 (F).
Figure 2
Figure 2
Histologic and immunohistochemical findings of blastic plasmacytoid dendritic cell neoplasm (BPDCN) of the 2nd case. (A) At low magnification, the nasopharyngeal mucosa is entirely replaced by diffuse atypical lymphoid cells microscopically. (B) Diffuse monomorphous infiltrate of medium-sized blast cells showing irregular nuclei with scanty cytoplasm. Immunohistochemically, these atypical lymphoid cells are positive for CD4 (C), weak CD56 (D), CD123 (E), and TCL1 (F).
See this image and copyright information in PMC

References

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    1. Facchetti F, Petrella T, Pileri SA. Swerdlow SHCE, Harris NL, et al. Blastic plasmacytoid dendritic cells neoplasm. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon France: IARC Press; 2017. 173–7.

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