a Departments of Medicine and Hematology , The Karolinska Institutet, Karolinska University Hospital Huddinge , Stockholm , Sweden.
b Center for Hematology and Regenerative Medicine (HERM), Departments of Hematology and Clinical Immunology and Transfusion Medicine , Karolinska University Hospital Huddinge , Stockholm , Sweden.
Expert Rev Hematol. 2016 May;9(5):479-87. doi: 10.1586/17474086.2016.1142867. Epub 2016 Feb 16.
Neutropenias (NPs), being acute and often transient, or chronic, range from life-threatening conditions with very low absolute neutrophil blood counts (ANC) to disorders characterized by only mild NP and of no obvious significance for health. Many are caused by genetic variations/mutations, e.g. the benign familial NP and the chronic severe NPs (e.g. Kostmann disease). Some of the latter are associated with various bodily malformations. Many of the mild-to-moderate NPs are signs of underlying disorders that need specialized treatments (e.g. HIV, hepatitis, autoimmune disorders, the large granular lymphocyte syndrome). We provide here means for the evaluation of a previously unknown NP, suggest a triage and treatments.
中性粒细胞减少症(NPs),无论是急性和常为一过性的,还是慢性的,范围从伴有非常低绝对中性粒细胞计数(ANC)的危及生命的病症,到仅表现为轻度中性粒细胞减少症且对健康无明显意义的病症。许多病症是由遗传变异/突变引起的,例如良性家族性中性粒细胞减少症和慢性严重中性粒细胞减少症(例如 Kostmann 病)。后者中的一些与各种身体畸形有关。许多轻度至中度的中性粒细胞减少症是潜在疾病的迹象,需要专门的治疗(例如 HIV、肝炎、自身免疫性疾病、大颗粒淋巴细胞综合征)。我们在此提供了一种用于评估先前未知的中性粒细胞减少症的方法,提出了一种分诊和治疗建议。