Brown R E, D'Cruz C A
Ann Clin Lab Sci. 1987 May-Jun;17(3):162-70.
Sinus histiocytosis massive lymphadenopathy (SHML) syndrome with hepatic involvement, occurring in a seven-year-old black female, is reported. Morphologic characterization of the hepatic lesion is accomplished utilizing conventional light, fluorescent and electron microscopy, and histochemical techniques and by comparing and contrasting the findings with those in cases of familial erythrophagocytic lymphohistiocytosis (FEL) and virus-associated hemophagocytic syndrome (VAHS). The histiocytic proliferation in the liver in SHML differs by showing: (a) an intralobular distribution with portal sparing; (b) marked steatosis; and (c) lipofuscinosis. The aforesaid intralobular distribution and the accompanying hypertrophy and hyperplasia of Kupffer cells, as well as commonalities of steatosis and lipofuscinosis, and, to a lesser extent, erythrophagocytosis and siderosis, suggest a histogenesis from Kupffer cells. The histochemical finding of fatty acid peroxides and both fluorescent microscopic and histochemical evidence of lipofuscin inclusions, a by-product of lipid peroxidation, in Kupffer cells provide at least a theoretical basis for both erythrophagocytosis and proliferation eventuating in intralobular histiocytosis.
本文报告了一名七岁黑人女性患伴有肝脏受累的窦组织细胞增生症伴巨大淋巴结病（SHML）综合征的病例。利用传统光学显微镜、荧光显微镜和电子显微镜、组织化学技术对肝脏病变进行形态学特征描述，并将结果与家族性噬血细胞性淋巴组织细胞增生症（FEL）和病毒相关噬血细胞综合征（VAHS）病例的结果进行比较和对比。SHML患者肝脏中的组织细胞增生表现为：（a）小叶内分布，不累及门脉区；（b）明显的脂肪变性；（c）脂褐素沉着。上述小叶内分布以及伴随的库普弗细胞肥大和增生，以及脂肪变性和脂褐素沉着的共性，以及在较小程度上的噬红细胞现象和含铁血黄素沉着，提示其起源于库普弗细胞。库普弗细胞中脂肪酸过氧化物的组织化学发现以及脂褐素包涵体的荧光显微镜和组织化学证据（脂质过氧化的副产物），为噬红细胞现象和导致小叶内组织细胞增生的增殖提供了至少一个理论基础。
