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17P deleted multiple myeloma presenting with intracranial disease: durable remission after tailored management
Joe Lee1, Dean Smith1, Neil Rabin1
1Clinical Haematology, University College London NHS Foundation Trust, London, UK.
Abstract:
In multiple myeloma (MM), del(17p) is associated with a poor outcome if present in greater than half the tumour cells. Similarly, intracranial involvement, often seen in the context of advanced disease, also heralds short survival. We present a rare case of MM presenting with intracranial disease and carrying del(17p) in 100% of tumour cells. This patient was successfully treated with combination chemotherapy employing central nervous system directed agents and bortezomib, followed by autologous stem cell transplant and consolidation with radiotherapy, bortezomib and thalidomide. We also present the outcomes of our single-centre experience of MM patients presenting with del(17p) disease. Copyright © 2015 John Wiley & Sons, Ltd.
Insights
This study details a rare case of multiple myeloma (MM) with significant del(17p) and brain involvement, successfully treated with chemotherapy and stem cell transplant. It also reviews outcomes for similar MM patients.
Area of Science:
- Hematology
- Oncology
- Genetics
Background:
- Multiple myeloma (MM) prognosis is significantly impacted by genetic aberrations like del(17p) and disease dissemination.
- Intracranial involvement in MM typically indicates advanced disease and poor survival outcomes.
Observation:
- A rare case of MM is presented, characterized by intracranial disease and del(17p) in 100% of tumor cells.
- This patient presented with a complex and aggressive form of multiple myeloma.
Findings:
- Successful treatment was achieved using a multi-modal approach including chemotherapy with CNS-directed agents, bortezomib, autologous stem cell transplant, and radiotherapy.
- The case highlights effective management strategies for advanced MM with high-risk genetic features.
Implications:
- This case and the accompanying single-center data offer valuable insights into managing aggressive multiple myeloma.
- Aggressive treatment regimens can lead to positive outcomes even in patients with poor prognostic markers like extensive del(17p).
