Mesh : Female Humans Middle Aged Neurolymphomatosis / diagnosis etiology Antineoplastic Combined Chemotherapy Protocols / therapeutic use Rituximab / therapeutic use Lymphoma, Large B-Cell, Diffuse / diagnosis drug therapy pathology Vincristine / therapeutic use Cyclophosphamide / therapeutic use Prednisone / therapeutic use Doxorubicin / therapeutic use

来  源:   DOI:10.1097/MD.0000000000037558   PDF(Pubmed)

Abstract:
BACKGROUND: Primary adrenal lymphoma (PAL) is a very rare and highly aggressive disease. Neurolymphomatosis (NL) is a rare manifestation of lymphoma characterized by the infiltration of lymphoma cells into peripheral nerves, resulting in neurological symptoms. To date, there have been very few reported cases of PAL with NL. By reviewing the entire treatment process of the patient, we aim to enhance recognition of PAL complicated with NL and guide clinicians to pay attention to the diagnosis of such diseases. Early recognition and diagnosis of NL are crucial for appropriate management and treatment decisions.
METHODS: We report a case of PAL in a 64-year-old female whose initial symptoms were pain and weakness in the left leg, which progressively worsened. In the half month before admission, the patient also showed signs of cranial nerve damage, such as diplopia and facial asymmetry.
METHODS: Computed tomography of the abdomen revealed an occupying lesion in the left adrenal region. Electromyography and somatosensory evoked potential examination of the extremities suggested left lumbar plexus damage and complete damage to the right facial nerve. Adrenal biopsy confirmed diffuse large B-cell lymphoma.
METHODS: The patient was treated with the R-CHOP scheme (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) combined with lenalidomide.
RESULTS: After 6 rounds of chemotherapy, the symptoms improved slightly. However, the condition progressed, and the patient passed away 1 year later.
CONCLUSIONS: Due to the nonspecific clinical presentation, patients with neurological damage should be alerted to the possibility of PAL and need to be evaluated thoroughly.
摘要:
背景:原发性肾上腺淋巴瘤(PAL)是一种非常罕见且高度侵袭性的疾病。神经淋巴瘤病(NL)是淋巴瘤的一种罕见表现,其特征是淋巴瘤细胞浸润到周围神经,导致神经症状.迄今为止,很少有报道的PAL伴NL的病例。通过回顾病人的整个治疗过程,我们旨在提高对PAL合并NL的认识,并指导临床医生注意此类疾病的诊断。NL的早期识别和诊断对于适当的管理和治疗决策至关重要。
方法:我们报告了一例PAL患者,其最初症状为左腿疼痛和无力,逐渐恶化。在入院前的半个月里,病人也有颅神经损伤的迹象,如复视和面部不对称。
方法:腹部计算机断层扫描显示左侧肾上腺区占位病变。四肢肌电图和体感诱发电位检查提示左腰丛损伤和右面神经完全损伤。肾上腺活检证实弥漫性大B细胞淋巴瘤。
方法:患者接受R-CHOP方案(利妥昔单抗,环磷酰胺,阿霉素,长春新碱,和泼尼松)联合来那度胺。
结果:经过6轮化疗,症状略有改善。然而,病情进展,患者一年后去世。
结论:由于非特异性临床表现,神经损伤患者应警惕PAL的可能性,并需要进行彻底评估。
公众号