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牛粒细胞病综合征的分子定义:Mac-1(CD11b/CD18)糖蛋白缺乏的鉴定

Molecular definition of the bovine granulocytopathy syndrome: identification of deficiency of the Mac-1 (CD11b/CD18) glycoprotein.

作者信息

Kehrli M E, Schmalstieg F C, Anderson D C, Van der Maaten M J, Hughes B J, Ackermann M R, Wilhelmsen C L, Brown G B, Stevens M G, Whetstone C A

机构信息

National Animal Disease Center, USDA-Agricultural Research Service, Ames, Iowa 50010.

出版信息

Am J Vet Res. 1990 Nov;51(11):1826-36.

PMID:1978618
Abstract

Leukocytosis (34,600 WBC/microliter of blood) was detected in an apparently healthy 7-day-old Holstein heifer. Analysis of blood samples obtained over the next 41 days revealed chronic progressive neutrophilia, which peaked at greater than 85% neutrophils and exceeded 100,000 WBC/microliter. In vitro assessment of isolated blood neutrophils obtained from the heifer at 38 and 45 days of age revealed selected functional abnormalities. Endocytosis of immunoglobulin-opsonized Staphylococcus aureus and killing of this test organism by the calf's neutrophils were significantly diminished, as were phagocytosis-associated superoxide generation, chemiluminescence activity, and myeloperoxidase-catalyzed iodination. Diminished H2O2 elaboration by the calf's neutrophils was evident during ingestion of opsonized zymosan or on exposure to phorbol myristate acetate. Extracellular release (secretion) of elastase during ingestion of zymosan was also diminished, although total cell content of elastase was normal, compared with that of neutrophils from age-matched calves, and granular or other morphologic abnormalities of the calf's neutrophils were not evident by ultrastructural examination. Abnormalities of random migration were inconsistently detected, and normal or high degree of antibody-dependent cytotoxicity or natural killing by the calf's neutrophils was observed. Similar in vitro assessment of neutrophils obtained from the calf's dam revealed no functional abnormalities. The calf died at 48 days of age, with persistent fever and chronic diarrhea, despite administration of antibiotics. Histologic examination at necropsy revealed large numbers of intravascular neutrophils in most tissues, including massive neutrophil sequestration in spleen. However, a striking lack of extravascular neutrophils was evident in inflamed submucosa adjacent to intestinal ulcers heavily contaminated with enteric microorganisms. Bone marrow examination revealed diffuse myeloid hyperplasia, but no other abnormalities.

摘要

在一头看似健康的7日龄荷斯坦小母牛中检测到白细胞增多症(血液白细胞计数为34,600/微升)。对接下来41天采集的血样分析显示存在慢性进行性中性粒细胞增多,中性粒细胞比例峰值超过85%,白细胞计数超过100,000/微升。对38日龄和45日龄小母牛分离出的血液中性粒细胞进行体外评估,发现了一些功能异常。小牛中性粒细胞对免疫球蛋白调理的金黄色葡萄球菌的内吞作用以及对该测试微生物的杀伤作用显著减弱,吞噬相关的超氧化物生成、化学发光活性以及髓过氧化物酶催化的碘化作用也减弱。在吞噬调理后的酵母聚糖或暴露于佛波酯肉豆蔻酸酯时,小牛中性粒细胞产生的过氧化氢明显减少。在吞噬酵母聚糖过程中弹性蛋白酶的细胞外释放(分泌)也减少,尽管与同龄小牛的中性粒细胞相比,弹性蛋白酶的总细胞含量正常,并且通过超微结构检查未发现小牛中性粒细胞有颗粒或其他形态异常。随机迁移异常检测结果不一致,并且观察到小牛中性粒细胞具有正常或高度的抗体依赖性细胞毒性或自然杀伤作用。对从小牛母亲处获得的中性粒细胞进行类似的体外评估,未发现功能异常。尽管使用了抗生素,这头小牛在48日龄时仍因持续发热和慢性腹泻而死亡。尸检时的组织学检查显示,大多数组织中有大量血管内中性粒细胞,包括脾脏中大量中性粒细胞滞留。然而,在被肠道微生物严重污染的肠道溃疡附近的炎症性黏膜下层明显缺乏血管外中性粒细胞。骨髓检查显示弥漫性髓样增生,但无其他异常。

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