Blastic plasmacytoid dendritic cell neoplasm: a rare case of gingival lesion with leukaemic presentation

Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare haematological malignancy with an aggressive clinical course. It has been recognised as a distinct entity in the WHO 2008 classification of haematolymphoid neoplasm. This disease usually presents with cutaneous involvement as the first manifestation, with subsequent or simultaneous spread to bone marrow and peripheral blood with leukaemic dissemination. Gingival lesion as the first manifestation, in the absence of a cutaneous lesion, is an uncommon presentation of this rare disease. We report a case of an elderly woman, aged 84 years, who presented with a lump on the mandibular gingiva and ipsilateral otalgia without any cutaneous lesion and associated with an highly aggressive and rapid leukaemia. This case is an addition to the handful of those cases of BPDCN which presents without cutaneous involvement, but it is exceedingly rare for clinical features that make it a unique case in the literature.

A rare case of Blastic plasmacytoid dendritic cell neoplasm with gynecologic presentation

“…Mucous lesions was been described in one case of gingival lesion [ 5 ]. Another case of gastric polypoid lesions was been reported [ 6 ].…”

Blastic Plasmacytoid Dendritic Cell Neoplasm: A Case Report of a Rare Leukemic Variant

“…While most BPCDN cases present with cutaneous lesions and multiple erythematous papules, the disease actually manifests as two clinically and pathologically distinct variants: the first and more common one presents with distinctive skin lesions, and with proliferating cells deriving from plasmacytoid dendritic cells precursors. The second variant is represented by nodular aggregates of clonally expanded plasmacytoid dendritic cells (DC) in lymph nodes, skin and bone marrow and a leukemic dissemination and presentation [4,5,8,9]. The latter is rare and affects predominantly males [9].…”

“…The second variant is represented by nodular aggregates of clonally expanded plasmacytoid dendritic cells (DC) in lymph nodes, skin and bone marrow and a leukemic dissemination and presentation [4,5,8,9]. The latter is rare and affects predominantly males [9]. Clinically, the course of BPDCN is characterized by a rapid progression to systemic disease via hematogenous dissemination, reflecting in an aggressive disease with poor outcome and a median overall survival ranging from 12 to 14 months [4,9].…”

“…The latter is rare and affects predominantly males [9]. Clinically, the course of BPDCN is characterized by a rapid progression to systemic disease via hematogenous dissemination, reflecting in an aggressive disease with poor outcome and a median overall survival ranging from 12 to 14 months [4,9]. Herein, we report a case of a patient with BPDCN with a rare leukemic variant of the disease at presentation.…”