Al-Hussain Turki, Hussein Maged H, Al Mana Hadeel, Akhtar Mohammed
*Department of Pathology and Laboratory Medicine †Department of Medicine, King Faisal Specialist Hospital and Research Center, Riyadh, Kingdom of Saudi Arabia.
Adv Anat Pathol. 2015 Mar;22(2):121-34. doi: 10.1097/PAP.0000000000000056.
Monoclonal gammopathy is produced by neoplastic or non-neoplastic expansion of a clone of plasma cells or B lymphocytes. Monoclonal gammopathy of unknown significance is characterized by low levels of the monoclonal protein and a relatively small population of clonal lymphocytes or plasma cells in the bone marrow. In these cases, the patient is asymptomatic with no evidence of overt myeloma or lymphoma. The abnormal serum protein may be present as a complete immunoglobulin molecule or may consist of ≥1 of its components such as light chains or heavy chains. These proteins may cause a variety of diseases in various tissues and organs, of which the kidney appears to be the most vulnerable. Renal involvement in monoclonal gammopathy may occur as part of a generalized disease such as amyloidosis, immunoglobulin deposition disease, and cryoglobulinemia. In addition, there may be evidence of kidney damage by processes which are renal specific. These include light chain proximal tubulopathy, light chain cast nephropathy, and a variety of glomerulopathies encompassing a wide spectrum of disease patterns.
单克隆丙种球蛋白病是由浆细胞或B淋巴细胞克隆的肿瘤性或非肿瘤性增殖产生的。意义未明的单克隆丙种球蛋白病的特征是单克隆蛋白水平低，骨髓中克隆性淋巴细胞或浆细胞群体相对较少。在这些病例中，患者无症状，没有明显骨髓瘤或淋巴瘤的证据。异常血清蛋白可能以完整免疫球蛋白分子形式存在，也可能由其≥1种成分组成，如轻链或重链。这些蛋白可能在各种组织和器官中引发多种疾病，其中肾脏似乎是最易受累的。单克隆丙种球蛋白病的肾脏受累可能是全身性疾病（如淀粉样变性、免疫球蛋白沉积病和冷球蛋白血症）的一部分。此外，可能有肾脏特异性过程导致肾损伤的证据。这些包括轻链近端肾小管病、轻链管型肾病以及涵盖广泛疾病模式的多种肾小球病。
