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A Rare Case of Epidural Myeloma Presenting as Recurrent Subdural Bleeding

Multiple myeloma (MM) is an incurable clonal B-cell malignancy that usually presents with neoplastic monoclonal plasma cells in either bone or soft tissues. Central nervous system involvement of the myeloma (CNS-MM), such as dural myeloma or intraparenchymal infiltration, or diffuse leptomeningeal i...

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Detalles Bibliográficos
Autores principales: Sivakolundu, Keerthana P, Vijaya Prakash, Aviraag, Savage, Natasha M, Kota, Vamsi K, Zarkua, Kristina
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8496494/
https://www.ncbi.nlm.nih.gov/pubmed/34660004
http://dx.doi.org/10.7759/cureus.17794
Descripción
Sumario:Multiple myeloma (MM) is an incurable clonal B-cell malignancy that usually presents with neoplastic monoclonal plasma cells in either bone or soft tissues. Central nervous system involvement of the myeloma (CNS-MM), such as dural myeloma or intraparenchymal infiltration, or diffuse leptomeningeal involvement, is uncommon. Dural involvement of myeloma without parenchymal or leptomeningeal disease is even rarer, with only seven cases reported previously. We present a case of epidural myeloma in a 50-year-old man with known kappa light chain MM, presenting with multiple episodes of subdural hemorrhage and progressive neurological deficits. He initially presented with severe back pain, hypercalcemia, and acute kidney injury (AKI). Further evaluation showed lytic bone lesions and elevated kappa light chains, and bone marrow biopsy showed 32% of clonal plasma cells. He was initially treated with bortezomib, lenalidomide, and dexamethasone combination, followed by pomalidomide and daratumumab. Eventually, he developed two episodes of subdural hemorrhage and left-sided seventh cranial nerve palsy, which was treated conservatively and monitored by computed tomography (CT) of the head. However, he gradually developed multiple cranial nerve palsies, weakness, and urinary incontinence. Cerebrospinal fluid (CSF) analysis showed elevated protein without any aberrant immunophenotype. Magnetic resonance imaging (MRI) of the brain showed diffuse smooth dural enhancement with extensive calvarial and skull base marrow replacement; MRI of the spine showed diffuse epidural enhancement in thoracic and lumbar regions, findings consistent with epidural myeloma. The patient received three doses of cranial irradiation but, unfortunately, could not tolerate further treatment and opted for hospice care. Intracranial hemorrhage is common in MM patients, and it is important to consider CNS involvement in patients presenting with recurrent subdural hemorrhage and to perform imaging (preferably MRI) earlier in the disease course. Due to its rarity, the treatment of CNS-MM is very heterogeneous. Thus, case reporting is important to accumulate data on this rare presentation.