Kohne E
Abteilung Kinderheilkunde II, Universitäts-Kinderklinik, Ulm.
Monatsschr Kinderheilkd. 1988 Dec;136(12):784-7.
In the paediatric patient neutropenia most often appears to be a transient symptom due to different non-haematological disorders. Persistent neutropenia, however, indicates a genuine disturbance of haemotopoiesis and myelopoiesis, respectively. The corresponding defects are either congenital or acquired, with clinical courses varying from asymptotic to life threatening disease. Diagnostic evaluation should comprise all methods available in order to obtain detailed classification of the type of neutropenic disorder. Therapeutic management is based on supprotive care. Immunosuppressive agents are discussed in patients with autoimmun neutropenia. For patients with severe congenital agranulocytosis bone marrow transplantation should be considered. In the future the availability of recombinant haematopoietic growth factors may provide a further therapeutic advantage.
在儿科患者中，中性粒细胞减少症通常似乎是由不同的非血液系统疾病引起的一种短暂症状。然而，持续性中性粒细胞减少症分别表明造血和骨髓生成存在真正的紊乱。相应的缺陷要么是先天性的，要么是后天获得的，临床病程从无症状到危及生命的疾病不等。诊断评估应包括所有可用方法，以便对中性粒细胞减少症的类型进行详细分类。治疗管理基于支持性护理。自身免疫性中性粒细胞减少症患者可讨论使用免疫抑制剂。对于严重先天性粒细胞缺乏症患者，应考虑进行骨髓移植。未来，重组造血生长因子的可用性可能会提供进一步的治疗优势。
