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.
- haematology (incl blood transfusion)
- haematology (drugs and medicines)
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Contributors AMK is the primary author of this case report. AMK identified the case, wrote the case report and did the literature review to extract relevant information for every section. AM made significant contribution to the case report, especially the discussion section by doing a thorough literature review. She was designated with the task of getting the radiology images and their description. MR is the haematologist of the patient in this case report. He helped us with the background of the patient and timeline of events. Since he was the one making the treatment decisions, he also provided us with the resources used by haematologists as a reference to treat this rare disease. He was a constant part of drafting and re-drafting to reach the final form of the case report. SM is a haematologist/oncologist. He helped with the editing and proofreading of the case report. He checked the accuracy of the material. Also, he gave the other case reports of this very rare disease which is used in the introduction and discussion.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.
Patient consent for publication Next of kin consent obtained.
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