Department of Internal Medicine and Clinical Immunology, Dijon University Hospital, 21079 Dijon, France.
Laboratory of Immunology, Dijon University Hospital, 21079 Dijon, France.
Eur J Intern Med. 2014 Nov;25(9):837-42. doi: 10.1016/j.ejim.2014.09.009. Epub 2014 Oct 11.
Although secondary hypogammaglobulinemia is more frequent than primary hypogammaglobulinemia, its etiology and management are poorly described, particularly for mild hypogammaglobulinemia.
This retrospective observational study included all adult patients with a gammaglobulin level <6.4g/L on serum electrophoresis identified at Dijon teaching hospital between April and September 2012. Clinico-biological features, etiologies and infectious complications were collected at inclusion and compared between group 1 (gammaglobulin <5g/L, severe hypogammaglobulinemia), and group 2 (gammaglobulin <6.4 and ≥5g/L, mild hypogammaglobulinemia).
Among the 4011 serum electrophoreses, 570 samples from 389 patients had gammaglobulin levels below 6.4g/L: 156 (40%) in group 1 and 233 (60%) in group 2. Mean age±SD was 67 (15) years, and sex ratio was 1.04 (M/F) with no difference between the two groups. An etiology was identified in 79% and 58% of patients in groups 1 and 2, respectively (p<0.0001). The main etiologies were similar in both groups and included malignant hemopathy treated with cytostatic agents (n=129, 33%), smoldering or newly-diagnosed hemopathy without treatment (n=49, 13%) and immunosuppressive treatment (n=91, 23%). The incidence of hypogammaglobulinemia-related infections was 22/100/year, with no significant difference between the two groups (p=0.17). Vaccination coverage against pneumococcus was 33%, and higher in group 1 (46% vs. 24%; p<0.0001). When no cause was known at inclusion, an etiology was discovered in 22/130 patients (17%), 11 in each group.
Though mild hypogammaglobulinemia does not meet the classical criteria for hypogammaglobulinemia (<5g/L), the etiology and infectious risk are similar. It therefore requires investigation and vaccination.
虽然继发性低丙种球蛋白血症比原发性低丙种球蛋白血症更为常见，但对其病因和治疗的描述较差，尤其是对于轻度低丙种球蛋白血症。
本回顾性观察性研究纳入了 2012 年 4 月至 9 月在第戎教学医院通过血清电泳发现的丙种球蛋白水平＜6.4g/L 的所有成年患者。在纳入时收集了临床生物学特征、病因和感染并发症，并在组 1（丙种球蛋白＜5g/L，严重低丙种球蛋白血症）和组 2（丙种球蛋白＜6.4 且≥5g/L，轻度低丙种球蛋白血症）之间进行了比较。
在 4011 份血清电泳中，570 份来自 389 名患者的丙种球蛋白水平＜6.4g/L：组 1 中为 156 例（40%），组 2 中为 233 例（60%）。两组的平均年龄±标准差分别为 67（15）岁和 1.04（M/F），性别比无差异。组 1 和组 2 中分别有 79%和 58%的患者确定了病因（p＜0.0001）。两组的主要病因相似，包括接受细胞毒性药物治疗的恶性血液病（n=129，33%）、未经治疗的冒烟型或新诊断的血液病（n=49，13%）和免疫抑制治疗（n=91，23%）。与低丙种球蛋白血症相关感染的发生率为 22/100/年，两组间无显著差异（p=0.17）。肺炎球菌疫苗覆盖率为 33%，组 1 更高（46%比 24%；p＜0.0001）。在纳入时无明确病因的情况下，在组 1 和组 2 中分别有 11 例和 11 例患者在纳入后发现病因（17%）。
尽管轻度低丙种球蛋白血症不符合低丙种球蛋白血症的经典标准（＜5g/L），但其病因和感染风险相似。因此，需要进行调查和接种疫苗。
