由抗NA1抗体引起的慢性自身免疫性中性粒细胞减少症。

Chronic autoimmune neutropenia due to anti-NA1 antibody.

作者信息

Valbonesi M, Campelli A, Marazzi M G, Cottafava F, Jannuzzi C

出版信息

Vox Sang. 1979;36(1):9-12. doi: 10.1111/j.1423-0410.1979.tb04391.x.

DOI:10.1111/j.1423-0410.1979.tb04391.x

Abstract

A 12-month-old child neutropenic since the age of 8 months, was referred to our institute for a sepsis from Candida albicans. On exploring the cause of neutropenia, an anti-NA1 antibody could be detected in the patient's serum. This antibody seemed to be responsible for the neutropenia because the child's PMN type was NA1+. The reactivity of the autoantibody with the patient's own granulocytes was confirmed by direct and indirect immunofluorescence studies performed on blood and marrow cells. A reduced number of T lymphocytes with poor PHA responsivity has been interpreted as the possible cause of the autoimmune disease. Steroid therapy did not cure the neutropenia but the child's general condition improved.

摘要

一名自8个月大起就患有中性粒细胞减少症的12个月大儿童，因白色念珠菌败血症被转诊至我院。在探究中性粒细胞减少症的病因时，在患者血清中检测到了一种抗NA1抗体。这种抗体似乎是中性粒细胞减少症的病因，因为患儿的中性粒细胞类型为NA1+。通过对血液和骨髓细胞进行直接和间接免疫荧光研究，证实了自身抗体与患者自身粒细胞的反应性。T淋巴细胞数量减少且对PHA反应性差被认为是自身免疫性疾病的可能病因。类固醇治疗未能治愈中性粒细胞减少症，但患儿的总体状况有所改善。