Nasr Samih H, Dogan Ahmet, Larsen Christopher P
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota;
Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, New York; and.
Clin J Am Soc Nephrol. 2015 Nov 6;10(11):2084-93. doi: 10.2215/CJN.12551214. Epub 2015 Apr 14.
Amyloidosis derived from leukocyte cell-derived chemotaxin 2 is a recently recognized form of amyloidosis, and it has already been established as a frequent form of systemic amyloidosis in the United States, with predominant involvement of kidney and liver. The disease has a strong ethnic bias, affecting mainly Hispanics (particularly Mexicans). Additional ethnic groups prone to develop amyloidosis derived from leukocyte cell-derived chemotaxin 2 include Punjabis, First Nations people in British Columbia, and Native Americans. Most patients are elderly who present with chronic renal insufficiency and bland urinary sediment. Proteinuria is variable, being absent altogether in about one third of patients. Liver involvement is frequently an incidental finding. Amyloidosis derived from leukocyte cell-derived chemotaxin 2 deposits shows a characteristic distribution: in the kidney, there is consistent involvement of cortical interstitium, whereas in the liver, there is a preferential involvement of periportal and pericentral vein regions. Concurrent renal disease is frequent, with diabetic nephropathy and IgA nephropathy being the most common. Patient survival is excellent, likely because of the rarity of cardiac involvement, whereas renal survival is guarded, with a median renal survival of 62 months in those without concurrent renal disease. There is currently no efficacious therapy for amyloidosis derived from leukocyte cell-derived chemotaxin 2 amyloidosis. Renal transplantation seems to be a reasonable treatment for patients with advanced renal failure, although the disease may recur in the allograft. The pathogenesis of amyloidosis derived from leukocyte cell-derived chemotaxin 2 amyloidosis has not yet been elucidated. It could be a result of leukocyte cell-derived chemotaxin 2 overexpression by hepatocytes either constitutively (controlled by yet-uncharacterized genetic defects) or secondary to hepatocellular damage. It is critical not to misdiagnose amyloidosis derived from leukocyte cell-derived chemotaxin 2 amyloidosis as Ig light chain-derived amyloidosis to avoid harmful chemotherapy.
源自白细胞衍生趋化因子2的淀粉样变性是一种最近才被认识的淀粉样变性形式，在美国它已被确认为全身性淀粉样变性的常见形式，主要累及肾脏和肝脏。该病具有强烈的种族倾向，主要影响西班牙裔（尤其是墨西哥人）。其他易患源自白细胞衍生趋化因子2的淀粉样变性的种族群体包括旁遮普人、不列颠哥伦比亚省的原住民和美洲原住民。大多数患者为老年人，表现为慢性肾功能不全和尿沉渣正常。蛋白尿情况不一，约三分之一的患者完全没有蛋白尿。肝脏受累常常是偶然发现。源自白细胞衍生趋化因子2的淀粉样变性沉积物有特征性分布：在肾脏，皮质间质始终受累，而在肝脏，门周和中央静脉区域优先受累。并发肾脏疾病很常见，糖尿病肾病和IgA肾病最为常见。患者生存率良好，可能是因为心脏受累罕见，而肾脏生存率情况不明，无并发肾脏疾病者的肾脏中位生存时间为62个月。目前对于源自白细胞衍生趋化因子2的淀粉样变性没有有效的治疗方法。肾移植似乎是晚期肾衰竭患者的合理治疗方法，尽管该病可能在同种异体移植中复发。源自白细胞衍生趋化因子2的淀粉样变性的发病机制尚未阐明。它可能是由于肝细胞组成性地（由尚未明确的基因缺陷控制）或继发于肝细胞损伤而导致白细胞衍生趋化因子2过度表达的结果。至关重要的是，不要将源自白细胞衍生趋化因子2的淀粉样变性误诊为Ig轻链衍生的淀粉样变性，以免进行有害的化疗。
