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低丙种球蛋白血症、意义未明的单克隆丙种球蛋白血症和体液免疫缺陷之间的关系:病例系列。

The relationship between hypogammaglobulinemia, monoclonal gammopathy of undetermined significance and humoral immunodeficiency: a case series.

机构信息

Allentown Asthma and Allergy, Allentown, PA 18104, USA.

出版信息

J Clin Immunol. 2011 Oct;31(5):737-43. doi: 10.1007/s10875-011-9548-0. Epub 2011 Jun 5.

DOI:10.1007/s10875-011-9548-0
PMID:21643891
Abstract

Hypogammaglobulinemia of the non-monoclonal immunoglobulin heavy chain classes has been reported in monoclonal gammopathy of undetermined significance (MGUS) patients. Whether low polyclonal immunoglobulin levels are associated with impaired specific antibody production and whether they represent a risk factor for the development of recurrent bacterial infections have not been established in this population. We determined the frequency of MGUS in patients referred to a tertiary care clinical immunology ambulatory care practice for evaluation of hypogammaglobulinemia, who were assessed for deficits in specific antibody production and the presence of recurrent infections. Of the 133 patients evaluated for hypogammaglobulinemia, 68 were screened for monoclonal gammopathy and 5 were found to have MGUS. Three had MGUS associated hypogammaglobulinemia in the absence of a defining primary immunodeficiency, one possibly had common variable immunodeficiency, and one had an uncertain diagnosis. Thus, MGUS may be uncovered in patients presenting with hypogammaglobulinemia even in those who lack an elevated serum level of IgG, IgM, or IgA.

摘要

在意义未明的单克隆丙种球蛋白血症(MGUS)患者中,已经报道了非单克隆免疫球蛋白重链类别的低丙种球蛋白血症。在该人群中,低多克隆免疫球蛋白水平是否与特异性抗体产生受损有关,以及它们是否代表复发性细菌感染的危险因素尚未确定。我们确定了在三级保健临床免疫学门诊就诊以评估低丙种球蛋白血症的患者中 MGUS 的发生率,这些患者接受了特异性抗体产生缺陷和复发性感染的评估。在评估低丙种球蛋白血症的 133 名患者中,有 68 名筛查了单克隆丙种球蛋白病,发现 5 名患有 MGUS。其中 3 名患有无明确原发性免疫缺陷的 MGUS 相关低丙种球蛋白血症,1 名可能患有普通可变免疫缺陷,1 名诊断不确定。因此,即使在那些血清 IgG、IgM 或 IgA 水平未升高的患者中,也可能在出现低丙种球蛋白血症的患者中发现 MGUS。

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Monoclonal gammopathy of undetermined significance (MGUS) and smoldering (asymptomatic) multiple myeloma: IMWG consensus perspectives risk factors for progression and guidelines for monitoring and management.意义未明的单克隆丙种球蛋白血症(MGUS)和冒烟型(无症状)多发性骨髓瘤:IMWG 共识观点——进展风险因素以及监测和管理指南。
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