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Longitudinal extensive transverse myelitis with an abnormal uFLC ratio in a pediatric patient: Case report and literature review

机译:小儿患者的uFLC比率异常的纵向广泛性横向脊髓炎:病例报告和文献复习

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Rationale: The serum and urine-free light chain (sFLC/uFLC) ratios of kappa (κ) to lambda (λ) serve as biomarkers for plasma cell disorders, especially multiple myeloma. However, to our best knowledge, the ratios have not been appropriately assessed for acute transverse myelitis (ATM). Patient concerns: We present a 12-year-old boy who had sudden onset low back pain following paralysis of his 4 extremities and disturbance consciousness. Magnetic resonance imaging (MRI) of the brain and spine indicated diffuse hyperintensity in T2-weighted images from the cervical spinal cord to the conus medullaris. An abnormal serum M-peak and uFLC ratio were detected in acute stage. Diagnoses: Based on the image findings, laboratory findings, and physical examination results, the diagnosis of acute transverse myelitis was established. Interventions and outcomes: With the treatment of pulse therapy and 5 courses of plasmapheresis, the patient had improvement in expanded disability status scale (EDSS) score from 9 to 5. Besides, the κ/λ ratio was also returned within the normal range. Lesson: The case presented an unusual phenomenon of transient abnormal κ/λ ratio combined with an M-peak in the acute course of longitudinally extensive transverse myelitis (LETM), which revealed FLC ratio recovering accompany with the improvement of disease. Further studies are required to identify the association between ATM and monoclonal gammopathy of undetermined significance (MGUS).
机译:理由:κ(κ)与λ(λ)的无血清和无尿轻链(sFLC / uFLC)比值可作为浆细胞疾病(尤其是多发性骨髓瘤)的生物标志物。但是,据我们所知,尚未对急性横贯性脊髓炎(ATM)的比率进行适当评估。患者关注:我们介绍了一个12岁的男孩,他的4个肢体瘫痪和意识障碍后突然发作了腰痛。大脑和脊柱的磁共振成像(MRI)表明,在T2加权图像中从颈脊髓到延髓的弥散性高信号。急性期血清M-peak和uFLC比值异常。诊断:根据影像学检查,实验室检查和身体检查结果,确定了急性横贯性脊髓炎的诊断。干预措施和结果:通过脉冲疗法和5个疗程的血浆置换术,患者的扩展残疾状态量表(EDSS)评分从9改善到5。此外,κ/λ比也恢复到正常范围。经验教训:该病例在纵向广泛性横断性脊髓炎(LETM)的急性过程中出现了一个短暂的异常κ/λ比值合并M峰的异常现象,这表明FLC比值的恢复伴随疾病的改善。需要进一步的研究来确定ATM与意义不明的单克隆丙种球蛋白病(MGUS)之间的关联。

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