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Blastic plasmacytoid dendritic cell neoplasm: a rare external ear lesion presenting with leukaemia
Hamad Dad Khan1, Sahil Kakar2, Lisha McClelland2
1Department of Otorhinolaryngology and Head and Neck Surgery, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK hamaddaadkhan@gmail.com.
Insights
This case study details a rare aggressive blood cancer, Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN), presenting uniquely as an ear swelling. Diagnosis required extensive investigation due to initial misleading symptoms.
Area of Science:
- Hematology
- Oncology
- Dermatology
Background:
- Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy.
- It typically presents with skin lesions and bone marrow involvement, posing diagnostic challenges.
Observation:
- A unique case of a woman in her 70s presented with recurrent ear swelling initially mimicking a hematoma.
- Initial diagnostic workup suggested vasculitis due to positive Myeloperoxidase (MPO) (p-ANCA) markers.
- Steroid treatment provided temporary improvement but was associated with an elevated white cell count (WCC).
Findings:
- Punch biopsy of the ear and bone marrow biopsy confirmed Blastic plasmacytoid dendritic cell neoplasm (BPDCN).
- The patient was also diagnosed with concurrent chronic myelomonocytic leukaemia.
- This case underscores the diagnostic complexity and varied presentations of BPDCN.
Implications:
- Highlights the importance of multidisciplinary collaboration in diagnosing rare hematologic malignancies.
- Expands the understanding of Blastic plasmacytoid dendritic cell neoplasm (BPDCN) presentations beyond typical cutaneous and bone marrow involvement.
- Emphasizes the need for thorough hematological review when initial treatments yield ambiguous results or unexpected changes occur.
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.
