Division of Hematology, University of British Columbia, Vancouver, BC, Canada; Department of Medicine, Vancouver General Hospital, Vancouver, BC, Canada.
Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, BC, Canada; Department of Medicine, Vancouver General Hospital, Vancouver, BC, Canada.
Lancet Haematol. 2021 May;8(5):e365-e375. doi: 10.1016/S2352-3026(21)00056-9.
This Review outlines a practical approach to assessing and managing polyclonal hypergammaglobulinaemia in adults. Polyclonal hypergammaglobulinaemia is most commonly caused by liver disease, immune dysregulation, or inflammation, but can also provide an important diagnostic clue of rare diseases such as histiocyte disorders, autoimmune lymphoproliferative syndrome, Castleman disease, and IgG4-related disease. Causes of polyclonal hypergammaglobulinaemia can be divided into eight categories: liver disease, autoimmune disease and vasculitis, infection and inflammation, non-haematological malignancy, haematological disorders, IgG4-related disease, immunodeficiency syndromes, and iatrogenic (from immunoglobulin therapy). Measuring serum concentrations of C-reactive protein and IgG subclasses are helpful in diagnosis. IL-6-mediated inflammation, associated with persistently elevated C-reactive protein concentrations (≥30 mg/L), is an important driver of polyclonal hypergammaglobulinaemia in some cases. Although the presence of markedly elevated serum IgG4 concentrations (>5 g/L) is around 90% specific for diagnosing IgG4-related disease, mildly elevated serum IgG4 concentrations are seen in many conditions. In most cases, managing polyclonal hypergammaglobulinaemia simply involves treating the underlying condition. Rarely, however, polyclonal hypergammaglobulinaemia can lead to hyperviscosity, requiring plasmapheresis.
这篇综述概述了一种评估和管理成人多克隆高丙种球蛋白血症的实用方法。多克隆高丙种球蛋白血症最常见的病因是肝脏疾病、免疫失调或炎症，但也可为组织细胞疾病、自身免疫性淋巴增生综合征、Castleman 病和 IgG4 相关疾病等罕见疾病提供重要的诊断线索。多克隆高丙种球蛋白血症的病因可分为八类：肝脏疾病、自身免疫性疾病和血管炎、感染和炎症、非血液系统恶性肿瘤、血液系统疾病、IgG4 相关疾病、免疫缺陷综合征和医源性（来自免疫球蛋白治疗）。测量血清 C 反应蛋白和 IgG 亚类浓度有助于诊断。IL-6 介导的炎症与持续升高的 C 反应蛋白浓度（≥30mg/L）相关，在某些情况下是多克隆高丙种球蛋白血症的重要驱动因素。虽然血清 IgG4 浓度显著升高（>5g/L）对 IgG4 相关疾病的诊断具有约 90%的特异性，但在许多情况下也会出现轻度升高的血清 IgG4 浓度。在大多数情况下，只需治疗潜在疾病即可治疗多克隆高丙种球蛋白血症。然而，极少数情况下，多克隆高丙种球蛋白血症可能导致高粘滞血症，需要血浆置换。
