DEFECTIVE NEUTROPHIL FUNCTION AND MICROBICIDAL MECHANISMS IN THE MYELODYSPLASTIC DISORDERS
DEFECTIVE NEUTROPHIL FUNCTION AND MICROBICIDAL MECHANISMS IN THE MYELODYSPLASTIC DISORDERS
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DOI：
10.1136/jcp.36.10.1120
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发表时间：
1983-01-01
影响因子：
3.4
通讯作者：
CHILD, JA
中科院分区：
文献类型：
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作者：
MARTIN, S;BALDOCK, SC;CHILD, JA
对44例原发性骨髓增生异常综合征(MDS)患者进行了中性粒细胞功能检测。对假丝酵母菌和金黄色葡萄球菌的吞噬和杀灭体外试验发现，13名患者出现中性粒细胞功能异常(白血病前期的迹象)，另有10名患者在病程中出现异常。5种疾病的功能缺陷发生率分别为：难治性细胞减少症(RC)8/17，难治性细胞减少伴铁粒母细胞改变(RC+SC)5/8，获得性特发性铁粒母细胞性贫血(AISA)2/4，难治性贫血伴原始细胞增多(RAEB)7/11，慢性粒单核细胞白血病(CMML)1/4。10 9/L和感染易感性被认为至少部分地反映了中性粒细胞的定性异常。中性粒细胞绝对计数与功能缺陷之间无相关性。中性粒细胞总体杀菌活性异常与吞噬功能受损和正常有关。部分细胞内杀伤缺陷患者髓过氧化物酶(MPO)活性降低，1例单磷酸己糖分流(HMPS)活性降低。2例中性粒细胞杀灭念珠菌活性明显受损的患者，在体外加入10-7M时，左旋咪唑纠正了功能，也在治疗剂量下给药。功能紊乱，可能反映了粒细胞系列成熟的异常，发生在骨髓增生异常的谱系中。几种杀微生物剂的机制可能存在缺陷。
Neutrophil function studies were carried out in 44 patients with primary myelodysplastic syndromes (MDS). In-vitro tests of phagocytosis and killing of Candida guilliermondii and Staphylococcus aureus identified 13 patients with abnormal neutrophil function [a sign of preleukemia] at presentation and a further 10 who developed abnormalities during the course of their disease. Incidence of defective function in the 5 disease categories was as follows: refractory cytopenia (RC) 8/17; refractory cytopenia with sideroblastic change (RC + SC) 5/8; acquired idiopathic sideroblastic anemia (AISA) 2/4; refractory anemia with excess blasts (RAEB) 7/11; chronic myelomonocytic leukemia (CMML) 1/4. Of 23 patients with defective neutrophil function, 11 experienced severe infective complications; in only 3 of these patients were neutrophil counts < 1 .times. 109/l and susceptibility to infection was considered to reflect, at least partially, qualitative neutrophil abnormalities. There was no correlation between absolute neutrophil count and defective function. Abnormal overall neutrophil microbicidal activity was equally associated with impaired and normal phagocytosis. Some patients with intracellular killing defects had reduced myeloperoxidase (MPO) activities and 1 had reduced hexose monophosphate shunt (HMPS) activity. In 2 patients, whose neutrophils showed markedly impaired candidacidal activity, levamisole corrected function when added in vitro at 10-7M and also when administered in therapeutic dosage. Deranged function, probably reflecting abnormalities in maturation of the granulocyte series, occurs across the myelodysplastic spectrum. Several microbicidal mechanisms may be defective.