A Family of Infantile Genetic Agranulocytosis
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概要
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This paper describes two siblings with Infantile genetic agranulocytosis (IGA). They had been susceptible to reccurent bacterial infections since early infancy. Peripheral blood showed agranulocytosis with variable monocytosis. Bone marrow revealed a tendency of maturation arrest at the promyelocytic or myelocytic level of granulopoiesis with concomitant increases in vacuolated monocytes. Consanguinity of the parents was noted. No antineutrophil antibody was found in the patient's as well as in mother's serum. The percentage of NBT positive monocytes in the peripheral blood was 50%, indicating that monocytosis might be a compensatory phenomenon for the paucity of neutrophils in defense. But the local cellular response (Skin window test) was hypocellular with monocytic infitrations 10 hours after abrasion. The markedly diminished accumulation of monocytes in inflammatory lesions seems to be an important cause of susceptibility to infection.
- 社団法人 日本内科学会の論文
社団法人 日本内科学会 | 論文
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