Zaidan Mohamad, Plasse Florent, Rabant Marion, Javaugue Vincent, Knebelmann Bertrand, Alyanakian Marie-Alexandra, Joly Dominique, Nochy Dominique, Bridoux Frank
Service de néphrologie-transplantation adultes, hôpital Necker, AP-HP, 149, rue de Sèvres, 75015 Paris, France; Université Paris Descartes, 12, rue de l'École-de-Médecine, 75006 Paris, France.
Service de néphrologie, CHU de Poitiers, 2, rue de la Milétrie, 86021 Poitiers, France; Centre national de référence amylose AL et autres maladies à dépôts d'immunoglobulines monoclonales, CHU de Poitiers, 2, rue de la Milétrie, 86021 Poitiers, France.
Nephrol Ther. 2016 Apr;12 Suppl 1:S71-81. doi: 10.1016/j.nephro.2016.01.010. Epub 2016 Mar 10.
Cryoglobulins are circulating immunoglobulins that precipitate with cold temperature and dissolve with rewarming. Type 1 cryoglobulinemia is composed of a single monoclonal immunoglobulin and is associated with renal involvement in up to 40% of cases. Type 1 cryoglobulinemia is related to an underlying B-cell haematological malignancy in 60% of patients. In the remaining cases, in the absence of criteria for malignancy, the diagnosis of monoclonal gammopathy of renal significance should be established. The clinical and biological setting and histological features of type 1 cryoglobulinemia are globally similar to those of mixed cryoglobulinemia. In case of haematological malignancy, the treatment is guided by the nature of the underlying disease, and aims at inducing haematological remission, which is necessary for the renal response. The management of monoclonal gammopathy of renal significance has been clarified by an international consensus group and is based on the nature of the underlying clone. In case of monoclonal cryoglobulinemia associated with a plasma-cell clone (IgG or IgA), the treatment is based on the combination of bortezomib, cyclophosphamide and dexamethasone. In case of IgM monoclonal cryoglobulinemia, the treatment is similar to that of Waldenström macroglobulinemia, and is based on rituximab. The clinical course of renal monoclonal cryoglobulinemia is intimately associated with the haematological response, and is usually favourable.
冷球蛋白是一种循环免疫球蛋白,在低温下沉淀,复温后溶解。1型冷球蛋白血症由单一单克隆免疫球蛋白组成,高达40%的病例会出现肾脏受累。60%的1型冷球蛋白血症患者与潜在的B细胞血液系统恶性肿瘤有关。在其余病例中,若不存在恶性肿瘤标准,则应确立具有肾意义的单克隆丙种球蛋白病的诊断。1型冷球蛋白血症的临床和生物学背景以及组织学特征总体上与混合性冷球蛋白血症相似。对于血液系统恶性肿瘤,治疗以潜在疾病的性质为指导,旨在诱导血液学缓解,这对肾脏反应是必要的。国际共识小组已明确了具有肾意义的单克隆丙种球蛋白病的管理方法,其基于潜在克隆的性质。对于与浆细胞克隆(IgG或IgA)相关的单克隆冷球蛋白血症,治疗基于硼替佐米、环磷酰胺和地塞米松的联合使用。对于IgM单克隆冷球蛋白血症,治疗与华氏巨球蛋白血症相似,基于利妥昔单抗。肾单克隆冷球蛋白血症的临床病程与血液学反应密切相关,通常预后良好。