Proliferative Glomerulonephritis with Monoclonal Immunoglobulin G Lambda Deposits: Report of the First Pediatric Case.
Proliferative Glomerulonephritis with Monoclonal Immunoglobulin G Lambda Deposits: Report of the First Pediatric Case.
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DOI：
10.1159/000488641
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发表时间：
2018-01
影响因子：
0.7
通讯作者：
Hendricks AR
中科院分区：
文献类型：
--
作者：
Torrealba J;Gattineni J;Hendricks AR
伴有单克隆免疫球蛋白G沉积的增殖性肾小球肾炎（PGNMID）是一种最近描述的罕见肾脏疾病，被认为是一种具有肾脏意义的单克隆免疫球蛋白病。虽然有些患者会有可检测的单克隆尖峰，明显的恶性血液病被发现，只有少数。大多数PGNMID患者年龄超过50岁，据我们所知，尚未报告儿童或青少年病例。肾活检显示光镜下不同的组织学模式，膜增生和膜模式是最常见的。免疫荧光显微镜显示限制为单一免疫球蛋白G重链同种型和单一轻链亚型。电子显微镜检查显示颗粒状、无组织的沉积物。我们报告一个罕见的儿科病例发生在一个17岁的女性。在成人人群中这种实体的罕见性还不允许建立标准的治疗方案。我们的青少年患者接受了多种治疗方案，包括泼尼松、吗替麦考酚酯、利妥昔单抗、硼替佐米和达雷妥尤单抗。我们的病例表明，儿科肾病学家和病理学家对这种疾病的认识对于指导准确的疾病分类、预后和治疗至关重要。
Proliferative glomerulonephritis with monoclonal immunoglobulin G deposits (PGNMID) is a recently described, uncommon renal disorder which is considered a monoclonal gammopathy of renal significance. Although some patients will have a detectable monoclonal spike, overt hematologic malignancy is found in only a minority. Most patients with PGNMID are over the age of 50 years, and to our knowledge no cases have been reported in children or adolescents. Renal biopsy shows variable histologic patterns by light microscopy, with membranoproliferative and membranous patterns being most common. Immunofluorescence microscopy demonstrates restriction to a single immunoglobulin G heavy chain isotype and a single light chain subtype. Electron microscopy reveals granular, unorganized deposits. We report a rare pediatric case which occurred in a 17-year-old female. The rarity of this entity in the adult population has not permitted a standard treatment regimen to be established. Our adolescent patient was treated with multiple treatment regimens including prednisone, mycophenolate mofetil, rituximab, bortezomib, and daratumumab. Our case demonstrates that awareness of this disorder by pediatric nephrologists and pathologists is vital to guide accurate disease classification, prognosis, and treatment.