Sivakolundu Keerthana P, Vijaya Prakash Aviraag, Savage Natasha M, Kota Vamsi K, Zarkua Kristina
Department of General Medicine, Government Kilpauk Medical College, Chennai, IND.
Department of Hematology and Oncology, Georgia Cancer Center at Augusta University, Augusta, USA.
Cureus. 2021 Sep 7;13(9):e17794. doi: 10.7759/cureus.17794. eCollection 2021 Sep.
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.
多发性骨髓瘤（MM）是一种无法治愈的克隆性B细胞恶性肿瘤，通常表现为骨骼或软组织中的肿瘤性单克隆浆细胞。骨髓瘤累及中枢神经系统（CNS-MM），如硬脊膜骨髓瘤或实质内浸润，或弥漫性软脑膜受累，并不常见。骨髓瘤仅累及硬脊膜而无实质或软脑膜病变的情况更为罕见，此前仅报道过7例。我们报告一例50岁男性硬膜外骨髓瘤病例，该患者已知患有κ轻链MM，表现为多次硬膜下出血和进行性神经功能缺损。他最初表现为严重背痛、高钙血症和急性肾损伤（AKI）。进一步评估显示溶骨性骨病变和κ轻链升高，骨髓活检显示32%的克隆性浆细胞。他最初接受硼替佐米、来那度胺和地塞米松联合治疗，随后接受泊马度胺和达雷妥尤单抗治疗。最终，他出现了两次硬膜下出血和左侧第七颅神经麻痹，对此进行了保守治疗，并通过头部计算机断层扫描（CT）进行监测。然而，他逐渐出现了多发性颅神经麻痹、虚弱和尿失禁。脑脊液（CSF）分析显示蛋白升高，但无任何异常免疫表型。脑部磁共振成像（MRI）显示硬脑膜弥漫性光滑强化，颅骨和颅底骨髓广泛被替代；脊柱MRI显示胸段和腰段硬膜外弥漫性强化，这些表现符合硬膜外骨髓瘤。患者接受了三剂头颅放疗，但不幸的是，无法耐受进一步治疗，选择了临终关怀。颅内出血在MM患者中很常见，对于出现复发性硬膜下出血的患者，重要的是考虑CNS受累情况，并在病程早期进行影像学检查（最好是MRI）。由于其罕见性，CNS-MM的治疗非常多样化。因此，病例报告对于积累关于这种罕见表现的数据很重要。
