An update on the diagnosis and treatment of chronic idiopathic neutropenia.
An update on the diagnosis and treatment of chronic idiopathic neutropenia.
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DOI：
10.1097/moh.0000000000000305
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发表时间：
2017-01
影响因子：
3.2
通讯作者：
Bolyard AA
中科院分区：
文献类型：
--
作者：
Dale DC;Bolyard AA
持续至少3个月且非药物或特定遗传、感染、炎症、自身免疫或恶性原因所致的中性粒细胞减少称为慢性特发性中性粒细胞减少。(CIN)CIN和自身免疫性中性粒细胞减少症（AIN）是非常相似和重叠的病症。临床结果取决于中性粒细胞减少症的严重程度，但不认为是癌前病变。对儿童的长期观察性研究表明，儿童该病通常持续3至5年，然后自发缓解，但成人病例很少缓解。抗中性粒细胞抗体测试在儿童和成人的价值是不确定的。最近的数据表明，CIN和AIN是免疫介导的疾病，但没有新的临床或基因检测来帮助诊断。用粒细胞集落刺激因子（G-CSF）治疗几乎在所有病例中都能有效增加血液中性粒细胞;然而，这种治疗仅适用于伴有中性粒细胞减少症和反复发热、炎症症状和感染的患者。很少或没有证据表明G-CSF治疗易使该人群发生髓系恶性肿瘤。重要的是要认识到CIN和AIN，这是儿童和成人慢性中性粒细胞减少症的最常见原因。如果中性粒细胞减少症不严重，即> 0.5 × 109/L，大多数患者可以观察到，不用抗生素或生长因子治疗。当中性粒细胞减少症严重时，G-CSF治疗通常是有益的。
Neutropenia lasting for at least for 3 months and not attributable to drugs or a specific genetic, infectious, inflammatory, autoimmune or malignant cause is called chronic idiopathic neutropenia. (CIN) CIN and autoimmune neutropenia (AIN) are very similar and overlapping conditions. The clinical consequences depend upon the severity of neutropenia, but it is not considered a premalignant condition. Long-term observational studies in children indicate that the disease often lasts for 3 to 5 years in children, then spontaneously remits, but it rarely remits in adult cases. The value of anti-neutrophil antibody testing in both children and adults is uncertain. Most recent data suggest that CIN and AIN are immune mediated diseases, but there are no new clinical or genetic tests to aid in diagnosis. Treatment with granulocyte colony stimulating factor (G-CSF) is effective to increase blood neutrophils in almost all cases; this treatment is reserved, however for patients with both neutropenia and evidence of recurrent fevers, inflammatory symptoms and infections. There is little or no evidence to indicate that G-CSF treatment predisposes to myeloid malignancies in this population. It is important to recognize CIN and AIN, the most common causes of chronic neutropenia in both children and adults. If the neutropenia is not severe, i.e. > 0.5 × 109/L, most patients can be observed and not treated prophylactically with antibiotics or a growth factor. When neutropenia is severe treatment with G-CSF is often beneficial.