大多数具有免疫活性体质的嗜中性白细胞减少症病人是由于循环血液中有直接抗特异性嗜中性白细胞抗原的自身抗体。通常,这些病人对皮质激素治疗虽有一定效果,但激素减量后常易复发。特发性血小板减少性紫癜亦存在类似情况。Imback等最近报导大剂量丙种球蛋白治疗自身免疫性嗜中性白细胞减少症儿童有效。有鉴于此,作者用此法治疗成年病人,这些病人每天用皮质激素维持正常或接近正常嗜中性白细胞计数,而且他们对血浆去除术效果亦极差。现举1例: 患者,女,25岁。因面部皮疹、进行性疲劳就诊。体检除口周痤疮样皮诊外,无明显异常。白细胞总数2,500,淋巴细胞89％,嗜中性白细胞7％,单核细胞4％。血常规生化检查正常,未测抗-DNA抗体。骨髓检查为正常骨髓细胞象。髓细胞:嗜酸性白细胞比率为4.5:1。未发现髓细胞样成熟停滞。软琼脂培养集落形成单位正常。肾上腺素刺激试验45分钟时嗜中性白细胞绝对值增加,从396增加到1,305。直接免疫荧 Most patients with an immunologically active neutropenia are autoantibodies that have a direct anti-specific neutrophil antigen in the circulating blood. Usually, these patients have some effect on the treatment of corticosteroids, but often relapse after hormone reduction. Idiopathic thrombocytopenic purpura also has a similar situation. Imback et al. Recently reported that high-dose gamma globulin is effective in children with autoimmune neutropenia. In view of this, the authors use this method to treat adult patients who have normal or near-normal neutrophil counts with corticosteroids daily and who are also poorly suited to plasmapheresis. Give an example: the patient, female, 25 years old. Due to facial rash, progressive fatigue treatment. Except for oral acne week examination, no obvious abnormalities. The total number of leukocytes 2,500, 89% of lymphocytes, 7% of neutrophils, monocytes 4%. Blood biochemical tests were normal, not tested anti-DNA antibodies. Bone marrow examination for normal bone marrow cells. Myeloid: eosinophil ratio was 4.5: 1. Myeloid-like maturation was not found. Soft agar culture colony formation unit normal. The neutrophil absolute value increased from 396 to 1,305 at 45 minutes of the adrenaline stimulation test. Direct immunofluorescence