Nakhleh R E, Glock M, Snover D C
Department of Laboratory Medicine and Pathology, University of Minnesota, Minneapolis.
Arch Pathol Lab Med. 1992 Jan;116(1):71-5.
We reviewed the hepatic pathology of seven cases of chronic granulomatous disease of childhood. All patients were male, with an age range of 5 to 41 years. Hepatic biopsy with drainage or wedge resection was performed in five cases to remove abscesses. Autopsy was performed in three cases. Presentation was typical of infection (fever, leukocytosis) with an elevated serum alkaline phosphatase level. Histologically, the most consistent feature was the presence of foamy macrophages that contained a finely granular golden brown pigment, seen in all seven cases. These were present as small collections predominantly in the portal tracts but were also found in the lobules. Palisading granulomas with central necrosis and associated giant cells were seen in four cases, one of which also had occasional lobular epithelioid granulomas. One case showed hyalinized portal and lobular granulomas. Four cases that showed palisading granulomas cultured positive for Staphylococcus aureus. One case cultured Pseudomonas cepacia, and one case cultured Streptococcus intermedius. Although palisading granulomas are typical of chronic granulomatous disease, they are not seen in all cases. These granulomas are similar to granulomas that are seen with Candida and other fungal infections and therefore are not specific for chronic granulomatous disease of childhood. The pigmented macrophages appear to be a consequence of the primary defect of the disease and are not secondary to infection and associated inflammation.
我们回顾了7例儿童慢性肉芽肿病的肝脏病理学表现。所有患者均为男性，年龄在5至41岁之间。5例行肝活检加引流或楔形切除术以清除脓肿。3例行尸检。临床表现为典型的感染症状（发热、白细胞增多），血清碱性磷酸酶水平升高。组织学上，最一致的特征是存在泡沫状巨噬细胞，其中含有细颗粒状金棕色色素，7例均可见。这些细胞以小聚集形式主要存在于汇管区，但也可见于肝小叶。4例可见伴有中央坏死和相关巨细胞的栅栏状肉芽肿，其中1例还偶见小叶上皮样肉芽肿。1例表现为玻璃样变的汇管区和小叶肉芽肿。4例出现栅栏状肉芽肿的患者金黄色葡萄球菌培养阳性。1例洋葱伯克霍尔德菌培养阳性，1例中间型链球菌培养阳性。尽管栅栏状肉芽肿是慢性肉芽肿病的典型表现，但并非所有病例均可见。这些肉芽肿与念珠菌及其他真菌感染时所见的肉芽肿相似，因此并非儿童慢性肉芽肿病所特有。色素沉着巨噬细胞似乎是该疾病原发性缺陷的结果，而非继发于感染及相关炎症。
