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伴有高球蛋白血症的慢性特发性中性粒细胞减少症。

Chronic idiopathic neutropenia with hypergammaglobulinemia.

作者信息

Watanabe N, Kobayashi M, Shigeta Y, Hara H

出版信息

Jpn J Med. 1987 Feb;26(1):65-8. doi: 10.2169/internalmedicine1962.26.65.

Abstract

Chronic idiopathic neutropenia can occur in spite of the normocellular bone marrow in myeloid series with or without anti-neutrophilic antibody. We report a patient with chronic neutropenia and severe polyclonal hypergammaglobulinemia. The patient demonstrated a positive anti-neutrophil antibody by fluorocytometry, although granulocyte-specific anti-nuclear factor and anti-stem cell (CFU-GM) antibody were negative. Thus, neutropenia of this patient appeared to be due to the antibody-mediated destruction of neutrophils. Both neutropenia and hypergammaglobulinemia were normalized by the administration of prednisone.

摘要

尽管骨髓中髓系细胞正常,无论有无抗中性粒细胞抗体,均可发生慢性特发性中性粒细胞减少症。我们报告了一例患有慢性中性粒细胞减少症和严重多克隆高球蛋白血症的患者。该患者通过荧光细胞术检测显示抗中性粒细胞抗体呈阳性,尽管粒细胞特异性抗核因子和抗干细胞(CFU-GM)抗体均为阴性。因此,该患者的中性粒细胞减少似乎是由于抗体介导的中性粒细胞破坏所致。给予泼尼松后,中性粒细胞减少症和高球蛋白血症均恢复正常。

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