Blastic plasmacytoid dendritic cell neoplasm in the background of myeloproliferative disorder and chronic lymphocytic leukaemia.
Aged
Antineoplastic Combined Chemotherapy Protocols
/ therapeutic use
Biopsy
CD4-Positive T-Lymphocytes
/ metabolism
CD56 Antigen
/ metabolism
Dendritic Cells
Diagnosis, Differential
Facial Neoplasms
/ drug therapy
Fatal Outcome
Female
Head and Neck Neoplasms
/ drug therapy
Humans
Leukemia, Lymphocytic, Chronic, B-Cell
/ complications
Rare Diseases
Scalp
Skin Neoplasms
/ drug therapy
Thrombocythemia, Essential
/ complications
chemotherapy
haematology (drugs and medicines)
haematology (incl blood transfusion)
oncology
radiology
Journal
BMJ case reports
ISSN: 1757-790X
Titre abrégé: BMJ Case Rep
Pays: England
ID NLM: 101526291
Informations de publication
Date de publication:
15 Jul 2019
15 Jul 2019
Historique:
entrez:
17
7
2019
pubmed:
17
7
2019
medline:
14
1
2020
Statut:
epublish
Résumé
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an extremely rare haematological malignancy defined by concurrent expression of CD4, CD56, BCL-2 and CD123. The disease has a very poor prognosis and there are no well-established treatment guidelines. We describe a case of BPDCN in a 65-year-old female patient with myeloproliferative disorder (essential thrombocythemia) and chronic lymphocytic leukaemia. She presented with rapidly progressive facial and scalp lesions. Skin biopsy confirmed BPDCN and the imaging revealed widespread disease. Patient was started on hyper-CVAD (cyclophosphamide, vincristine, doxorubicin, dexamethasone) and intrathecal methotrexate. Due to progression on initial treatment, she was treated with decitabine and venetoclax (BCL-2 inhibitor). However, patient continued to deteriorate and died after 4 months from initial diagnosis. We emphasise on the clinical features, emerging treatment modalities and prognosis of BPDCN.
Identifiants
pubmed: 31308179
pii: 12/7/e230332
doi: 10.1136/bcr-2019-230332
pmc: PMC6663200
pii:
doi:
Substances chimiques
CD56 Antigen
0
Types de publication
Case Reports
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Informations de copyright
© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.
Déclaration de conflit d'intérêts
Competing interests: None declared.
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