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Ear, nose and throat
Blastic plasmacytoid dendritic cell neoplasm: a rare external ear lesion presenting with leukaemia
  1. Hamad Dad Khan1,
  2. Sahil Kakar1,
  3. Lisha McClelland1 and
  4. Hayder Hussein2
  1. 1Department of Otorhinolaryngology and Head and Neck Surgery, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
  2. 2Department of Haematology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
  1. Correspondence to Mr Hamad Dad Khan; hamaddaadkhan{at}gmail.com

Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive haematological malignancy, typically characterised by cutaneous lesions and bone marrow involvement. We present a unique case of a woman in her 70s, initially seen for a spontaneous swelling on her left external ear resembling a haematoma, which recurred after initial treatment, triggering further evaluation.

Diagnostic challenges arose as the patient displayed positive markers for Myeloperoxidase (MPO) (p-ANCA), suggesting vasculitis. Dermatology considered various differential diagnoses, but imaging and tests ruled out significant pathology. Steroid treatment led to improvement, but coincided with a surge in white cell count (WCC), prompting an urgent haematological review.

Subsequent investigations, including a punch biopsy of the external ear and a bone marrow biopsy revealed BPDCN concurrent with chronic myelomonocytic leukaemia. This case highlights the challenging diagnostic journey, emphasising the need for multidisciplinary collaboration and the potential for unique BPDCN presentations, expanding our understanding of this malignancy.

  • Ear, nose and throat/otolaryngology
  • Haematology (incl blood transfusion)
  • Oncology
  • Chemotherapy

https://doi.org/10.1136/bcr-2024-260059

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    Footnotes

    • Contributors The following authors were responsible for drafting of the text, sourcing and editing of clinical images, investigation results, drawing original diagrams and algorithms, and critical revision for important intellectual content: HK, SK, LM, HH. The following authors gave final approval of the manuscript: LM, HH. AI used to check grammar and rephrase sentences.

    • 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.

    • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.