肝 ALECT-2 淀粉样变导致门静脉高压和复发性静脉曲张出血：病例报告及文献复习。
Hepatic ALECT-2 amyloidosis causing portal hypertension and recurrent variceal bleeding: a case report and review of the literature.
机构信息
Royal Victoria Hospital, Division of Hematology, 687 des pins, C6.82, Montreal, Quebec, Canada H3A 1A1;
出版信息
Am J Clin Pathol. 2014 Feb;141(2):288-91. doi: 10.1309/AJCPCLK54RKXTRDI.
OBJECTIVES
To examine laser microdissection and mass spectrometry (LMD-MS), which has emerged as a new tool to aid in typing amyloid proteins.
RESULTS
ALECT-2 is a potential cause of hepatic amyloidosis best detected by LMD-MS.
METHODS
One of the more recently reported proteins is ALECT-2 (leukocyte cell-derived chemotaxin 2) amyloid, found in renal specimens of Hispanic patients. Here we report the first case of hepatic ALECT-2 amyloidosis diagnosed by LMD-MS from a liver biopsy specimen of a 52-year-old Hispanic man and causing portal hypertension with recurrent esophageal variceal bleeding.
CONCLUSIONS
ALECT-2 can cause amyloidosis in organs other than the kidneys. It should be strongly considered in Hispanic patients and in those with a globular pattern of amyloid deposition. The incidence of ALECT-2 amyloidosis is likely underreported.
目的
探讨激光显微切割和质谱分析（LMD-MS），这是一种新的辅助淀粉样蛋白分型的工具。
结果
ALECT-2 是一种潜在的肝淀粉样变性病因，最好通过 LMD-MS 检测。
方法
最近报道的一种蛋白质是 ALECT-2（白细胞衍生趋化因子 2）淀粉样蛋白，在西班牙裔患者的肾标本中发现。在这里，我们报告了首例通过 LMD-MS 从 52 岁西班牙裔男性肝活检标本中诊断的肝 ALECT-2 淀粉样变性病例，该患者因门脉高压伴复发性食管静脉曲张出血。
结论
ALECT-2 可导致除肾脏以外的器官发生淀粉样变性。在西班牙裔患者和淀粉样蛋白沉积呈球形模式的患者中应强烈考虑该疾病。ALECT-2 淀粉样变性的发病率可能被低估。
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