Globular Hepatic Amyloid Is Highly Sensitive and Specific for LECT2 Amyloidosis
Globular Hepatic Amyloid Is Highly Sensitive and Specific for LECT2 Amyloidosis
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DOI：
10.1097/pas.0000000000000373
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发表时间：
2015-04-01
影响因子：
5.6
通讯作者：
Wu, Tsung-Teh
中科院分区：
文献类型：
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作者：
Chandan, Vishal S.;Shah, Sejal S.;Wu, Tsung-Teh
球状肝淀粉样蛋白（GHA）是罕见的，其临床意义仍不清楚。最近，白细胞趋化因子相关的淀粉样变性（ALECT 2）已被报道涉及肝脏，显示出一个球状的模式。我们回顾了70例连续的肝淀粉样变性病例，以确定肝淀粉样蛋白亚型的患病率和形态，特别是ALECT 2及其与GHA的关系。对每个病例的淀粉样蛋白亚型（免疫组织化学和/或质谱法）、其模式（线性或球形）和分布（血管、窦周或基质）进行审查。此外，我们还审查了24例经质谱法证实的肝脏ALECT 2病例。49例进行了LECT 2免疫组化染色。其中免疫球蛋白轻链（AL）型41例（59%），甲状腺素运载蛋白（ATTR）型15例（22%），纤维蛋白原A（AFib）、血清淀粉样蛋白A（AA）、ALECT 2型各3例（4%），载脂蛋白（AApoA 1）型2例（3%），未分型3例（4%）。我们的70例ALECT 2病例中的3例（4%）和所有24例（100%）经质谱证实的肝ALECT 2病例仅显示肝窦和汇管区中的GHA沉积。其他3例（4%）AL型病例显示局灶性球形图案，混合有明显的线性淀粉样蛋白。其他淀粉样蛋白亚型均未显示GHA。27例ALECT 2阳性（100%），22例非ALECT 2阳性（100%）（AL 14例，ATTR 5例，AA 1例，AFib 1例，AApoA 1 1例）。纯GHA是罕见的（4%），但对ALECT 2高度特异性，LECT 2免疫染色有助于确认这种淀粉样蛋白类型。
Globular hepatic amyloid (GHA) is rare, and its clinical significance remains unclear. Recently, leukocyte chemotactic factor-associated amyloidosis (ALECT2) has been reported to involve the liver, showing a globular pattern. We reviewed 70 consecutive cases of hepatic amyloidosis to determine the prevalence and morphology of hepatic amyloid subtypes, especially ALECT2 and its association with GHA. Each case was reviewed for amyloid subtype (immunohistochemistry and/or mass spectrometry), its pattern (linear or globular), and distribution (vascular, perisinusoidal, or stromal). In addition, 24 cases of confirmed hepatic ALECT2 on mass spectrometry from our consultation files were also reviewed. LECT2 immunostaining was performed in 49 cases. Of the 70 cases, immunoglobulin light chain (AL) type was most common with 41 cases (59%), followed by transthyretin (ATTR) 15 cases (22%), 3 cases each of fibrinogen A (AFib) (4%), serum amyloid A (AA) (4%), and ALECT2 (4%), 2 cases of apolipoproteins (AApoA1) (3%), and 3 cases (4%) were unclassified. Three of our 70 cases (4%), with ALECT2, and all 24 cases (100%) of mass spectrometry-confirmed hepatic ALECT2 showed only GHA deposits in the hepatic sinusoids and portal tracts. Three (4%) other cases of AL type showed a focal globular pattern admixed with prominent linear amyloid. None of the other amyloid subtypes showed GHA. LECT2 immunostain was positive in all 27 cases (100%) of ALECT2 and negative in the other 22 non-ALECT2 cases (100%) (14 AL, 5 ATTR, 1 AA, 1 AFib, 1 AApoA1). Pure GHA is uncommon (4%) but is highly specific for ALECT2, and LECT2 immunostain is helpful in confirming this amyloid type.