Abstract
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.
摘要
球状肝淀粉样变(GHA)非常罕见,而其临床意义还不明确。最近,有报道称白细胞趋化因子相关淀粉样变(ALECT2) 可以累及肝脏,呈球状结构。我们连续复习了70例肝淀粉样变的病例以了解肝淀粉样变各种亚型的患病率及其形态特点,尤其是ALECT2 及其与球状肝淀粉样变的联系。我们对每一病例都进行了淀粉样变亚型的划分(通过免疫组化或肿物光谱测定法),复习了其结构模式(线状或球状)及其分布(血管,窦周,或间质)。此外,还回顾了我们会诊档案中24例经肿块光谱测定法确诊的肝脏ALECT2病例。我们对49例病例进行了LECT2的免疫组化染色。70例中,免疫球蛋白轻链型 (AL)最常见,有41例(59%),其次是甲状腺素运载蛋白 (ATTR)有15例(22%),3例纤维蛋白原A(AFib) (4%),血清淀粉样蛋白A(AA) (4%), ALECT2 (4%),2例载脂蛋白 (AApoA1) (3%), 3例(4%)未分类。我们的70例病例中有3例 (4%)伴有ALECT2和所有24例 (100%) 经肿物光谱测定法证实的肝ALECT2显示GHA 沉积于肝血窦和汇管区。AL型中有3例 (4%) 显示局限性结节状混合突出的线状淀粉样变。其他亚型的淀粉样变中均未见GHA。LECT2的免疫染色在所有27例 (100%)ALECT2病例中均为阳性,在其他22例 (100%)非ALECT2 (14 AL, 5 ATTR, 1 AA, 1 AFib, 1 AApoA1)病例中均为阴性.单纯性GHA少见(4%)却是高度特异的ALECT2,LECT2的免疫染色有助于证实这类型的淀粉样变。
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