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Pelger-Huet白细胞畸形(简称PHA)表现为周围血中粒细胞核分叶不完全,核染色质较正常粗糙而凝聚,属常染色体显性遗传,主要为杂合子型,纯合子型罕见并伴有高度的早期死亡率,死亡可能由感染所致。我校于1983年3月发现一女学生周围血中出现粒细胞核不分叶,经家系调查,在本家系成员24人中发现14例 Pelger-Huet白细胞畸形,现报告如下: 患者女,21岁,汉族,于1983年3月10日查白细胞5600,中幼粒0.5％,晚幼粒1％,嗜酸性晚幼粒1.5％,嗜酸性杆状核2％,中性杆状核65.5％,2叶核7％,未见3叶核细胞。淋巴细胞25％,单核细
Pelger-Huet white blood cell deformity (referred to as PHA) showed peripheral blood neutrophil lobulation is not complete, nuclear chromatin than normal cohesion, is an autosomal dominant inheritance, mainly heterozygous homozygous rare and associated with High levels of early mortality, death may be caused by the infection. My school in March 1983 found that a female students around the blood in the presence of granulocytes without lobes, the pedigree investigation, found in the family of 24 members of 14 cases of Pelger-Huet leukocyte deformity, are reported as follows: Female patients, 21 years old , Han nationality, on March 10, 1983 check white blood cells 5600, 0.5% in the young, late promyelocytic 1%, 1.5% of eosinophils, eosinophilic corpuscles 2%, 65.5% 2 leaf 7%, no 3 leaf cells. 25% of lymphocytes, mononuclear