Stručni rad
Blastic Plasmacytoid Dendritic Cell Neoplasm, a Very Rare Hematological Malignancy With Initial Cutaneous Involvement: A Case Report
Jelena Lalošević Mišović
; Clinical Hospital Center “Zvezdara”, Belgrade, Serbia;
Jovan Lalošević
orcid.org/0000-0003-4830-7514
; University of Belgrade School of Medicine, Belgrade, Serbia
Mirjana Gajić-Veljić
; University of Belgrade School of Medicine, Belgrade, Serbia
Tatjana Terzić
; University of Belgrade School of Medicine, Belgrade, Serbia
Miloš Nikolić
orcid.org/0000-0002-3926-5394
; University of Belgrade School of Medicine, Belgrade, Serbia
Sažetak
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a very rare and aggressive hemato-
logic malignancy, arising from plasmacytoid dendritic cells (pDCs). BPDCN frequently has,
at least initially, exclusively cutaneous presentation.
We present a 45-year-old woman with a 3-month history of rapidly evolving violaceous
patches, infiltrated plaques, and bruise-like tumefactions, disseminated on her face and up-
per trunk. Histopathology of a lesion, along with Flow cytometry of peripheral blood and
cerebrospinal fluid, confirmed the diagnosis of BPDCN. The patient received a hyper-CVAD
(cyclophosphamide, vincristine, doxorubicin, dexamethasone) regimen and six triple intra-
thecal chemotherapies. Unfortunately, the patient contracted COVID-19 and died of severe
respiratory complications, despite all the available treatment modalities.
Our patient presented with typical clinicopathological manifestations of the disease, mis-
diagnosed for 3 months. The case additionally reflects difficulties in patient management
during the COVID-19 pandemic.
Dermatologists should be aware of this rare disease, since the early diagnosis and treat-
ment with new emerging drugs may lead to a better prognosis.
Ključne riječi
blastic plasmacytoid dendritic cell neoplasm (BPDCN); skin, dendritic cells; leukemia; hematologic neoplasm, CD123
Hrčak ID:
326851
URI
Datum izdavanja:
10.4.2024.
Posjeta: 568 *