Nasim SALIMIAGHDAM, Usha NIRANJAN
Introduction. Multiple myeloma (MM) is a type of cancer that affects plasma cells and makes up about 10% of blood cancer. It’s characterized by uncontrolled growth of plasma cells in the bone marrow, which often leads to excess monoclonal immunoglobulins or free light chains. Lambda light chain multiple myeloma (MM) is a specific type of light chain MM, accounting for roughly 15–20% of all MM cases, and it tends to exhibit more aggressive clinical features and organ involvement.1,2 Involvement in multiple myeloma is exceptionally rare, accounting for less than 1% of extramedullary presentations, and is typically associated with aggressive disease biology.3
Several cytogenetic abnormalities—most consistently gain(1q21), del(17p), and t(4;14)- are well-established adverse prognostic markers associated with inferior progression-free and overall survival. The impact of some additional risk features may vary by treatment era and regimen intensity, emphasizing the need to interpret prognostic markers alongside contemporary induction, transplant, and maintenance strategies [4]. Extramedullary disease (EMD) is heterogeneous (e.g., soft-tissue plasmacytomas vs bone-associated lesions) and lacks standardized treatment protocols. While EMD may respond less reliably to conventional approaches, the evidence base remains limited, and responses can vary with newer agents, including anti-CD38 antibodies and evolving cellular therapies.5
This report details a particularly complex case of lambda light-chain MM presenting with rare orbital involvement and intracranial extension, features that underscore the clinical rarity and high-risk nature of extramedullary spread and highlight the need for multimodal therapy, including early radiotherapy. The case highlights the diagnostic challenges posed by unusual presentations and highlights the growing importance of new treatment options in high-risk MM.
Keywords: Multiple myeloma, lambda light chain, orbital mass, 1q21 gain, extramedullary involvement, stem cell transplant, bortezomib, daratumumab, Revlimid
https://doi.org/10.59854/dhrrh.2026.4.3.133
Cite this article
Salimiaghdam N., Niranjan U., When the Eyes Speak: A Multisystem Challenge with CNS and Orbital Spread Light Chain Myeloma. DHRRH, 2026, 6(2), https://doi.org/10.59854/dhrrh.2026.4.3.133
