Lazaro Estibaliz, Morel Jacques
Service de médecine interne, hôpital du Haut-Lévêque, université de Bordeaux, 33604 Pessac, France.
Service de médecine interne, hôpital du Haut-Lévêque, université de Bordeaux, 33604 Pessac, France; Département de rhumatologie, hôpital Lapeyronie, université de Montpellier 1, 34295 Montpellier cedex 5, France.
Joint Bone Spine. 2015 Jul;82(4):235-9. doi: 10.1016/j.jbspin.2015.01.005. Epub 2015 Mar 26.
Neutropenia is defined as a neutrophil count lower than 1.5g/L, with categorization as mild, moderate, or severe when the count is 1.5-1g/L, 1-0.5g/L, or<0.5g/L, respectively. The main complication is infection, whose risk increases with the depth and duration of the neutropenia. Comprehensive etiological investigations are mandatory to determine the best treatment strategy. Constitutional neutropenia is rarely seen in everyday rheumatology practice. It predominantly affects patients of African descent and is usually moderate and well tolerated. Congenital neutropenia due to genetic abnormalities is severe and chiefly seen in the pediatric population. Most cases of neutropenia in patients with rheumatoid arthritis (RA) are acquired. Medications are the most common causes, making detailed history-taking crucial. Many medications used to treat RA can induce neutropenia. Folic acid deficiency should be sought routinely in patients taking methotrexate. A less common cause of neutropenia is an RA-related autoimmune reaction. Splenomegaly suggests Felty's syndrome, which is accompanied with large granular lymphocytic (LGL) leukemia in 40% of cases. The treatment depends on the depth of the neutropenia and findings from the etiological workup. A neutrophil count below 0.5g/L, a fever, and the presence of clinical signs indicate a life-threatening condition requiring emergent treatment. In other patients, the first step is immediate discontinuation of any possibly involved drugs, simultaneously with the etiological workup.
中性粒细胞减少症定义为中性粒细胞计数低于1.5g/L，当计数分别为1.5 - 1g/L、1 - 0.5g/L或<0.5g/L时，分为轻度、中度或重度。主要并发症是感染，其风险随着中性粒细胞减少的程度和持续时间增加。必须进行全面的病因调查以确定最佳治疗策略。在日常风湿病学实践中很少见到体质性中性粒细胞减少症。它主要影响非洲裔患者，通常为中度且耐受性良好。由于基因异常导致的先天性中性粒细胞减少症较为严重，主要见于儿科人群。类风湿关节炎（RA）患者的中性粒细胞减少症大多是后天获得性的。药物是最常见的原因，因此详细的病史采集至关重要。许多用于治疗RA的药物可诱发中性粒细胞减少症。服用甲氨蝶呤的患者应常规检查是否缺乏叶酸。中性粒细胞减少症的一个较不常见的原因是与RA相关的自身免疫反应。脾肿大提示费尔蒂综合征，40%的病例伴有大颗粒淋巴细胞（LGL）白血病。治疗取决于中性粒细胞减少的程度和病因检查结果。中性粒细胞计数低于0.5g/L、发热以及出现临床体征表明存在危及生命的情况，需要紧急治疗。在其他患者中，第一步是立即停用任何可能相关的药物，同时进行病因检查。
