目的:在一项前瞻性研究中评估慢性特发性和自身免疫性中性粒细胞减少症成年患者的感染和流行病学演变。
方法:从2008年9月至2012年4月,共纳入76例连续患者。在登记时进行完整的血细胞计数和临床评估,在第3、6个月,然后每6个月。抗中性粒细胞抗体用GIFT法检测。
结果:患者(49例慢性特发性和27例自身免疫性中性粒细胞减少症)的随访时间中位数为5年(范围24-84个月)。在入学时,44例患者中性粒细胞减少轻度(中性粒细胞中位数1.27×10(3)/μL),23中度(中位数0.8×10(3)/μL),9例严重(中位数0.4×10(3)/μL)。中性粒细胞计数显示出很大的受试者间差异,但没有受试者间差异,自身免疫性中性粒细胞减少症的价值较低,在男性中,在MGUS案例中。随着时间的推移,没有>3级感染发生;13/49慢性特发性和6/27自身免疫性中性粒细胞减少患者经历2级事件,无论中性粒细胞的平均值和最低点值。登记时的骨髓评估显示23%的病例细胞减少,和55%的红细胞生成异常特征,没有明确的血液学诊断.在后续行动中,5例诊断为NK细胞扩张,4患有毛细胞白血病,3例骨髓增生异常(1例粒单核细胞白血病,1例难治性血细胞减少伴单科发育不良,和1个多谱系发育不良),进化的中位时间为30个月。
结论:慢性特发性和自身免疫性中性粒细胞减少症,虽然通常是良性的,值得血液学随访,在诊断时进行骨髓评估,并在中性粒细胞减少症恶化的情况下进行重新评估,出现额外的血细胞减少症,和淋巴细胞增多。
OBJECTIVE: To evaluate infections and oncohematologic evolution in adult patients with chronic idiopathic and autoimmune neutropenia in a prospective
study.
METHODS: 76 consecutive patients were enrolled from September 2008 to April 2012. Complete blood counts and clinical evaluation were performed at enrolment, at month 3, 6, and then every 6 months. Anti-neutrophil antibodies were tested by GIFT method.
RESULTS: Patients (49 chronic idiopathic- and 27 autoimmune neutropenia) were followed for a median of 5 years (range 24-84 months). At enrolment, neutropenia was mild in 44 patients (median neutrophils 1.27×10(3)/μL), moderate in 23 (median 0.8×10(3)/μL), and severe in 9 (median 0.4×10(3)/μL). Neutrophil counts showed a great inter-subject but no intra-subject variability, with lower values in autoimmune neutropenia, in males, and in MGUS cases. Over time, no grade >3 infections occurred; 13/49 chronic idiopathic and 6/27 autoimmune neutropenia patients experienced a grade 2 event, irrespective of mean and nadir neutrophil values. Bone marrow evaluation at enrolment showed reduced cellularity in 23% of cases, and dyserythropoietic features in 55%, with no definite hematologic diagnosis. During the follow-up, 5 cases were diagnosed with NK expansion, 4 with hairy cell leukemia, and 3 with myelodysplasia (1 myelomonocytic leukemia, 1 refractory cytopenia with unilineage dysplasia, and 1 multilineage dysplasia), with a median time to evolution of 30 months.
CONCLUSIONS: Chronic idiopathic and autoimmune neutropenia, although usually benign, deserve hematological follow-up with a bone marrow evaluation at diagnosis and a re-evaluation in the presence of worsening neutropenia, appearance of additional cytopenias, and lymphocytosis.