Sorbonne universités, UPMC Université Paris 06, Inserm, UMR S 959, immunology immunopathology immunotherapy (I3), 75005, Paris, France; Biotherapy (CIC-BTi) and inflammation-immunopathology-biotherapy department (DHU i2B), hôpital Pitié-Salpêtrière, AP-HP, 75651, Paris, France; AP-HP, groupe hospitalier Pitié-Salpêtrière, department of internal medicine and clinical immunology, centre national de référence maladies autoinflammatoires et amylose inflammatoire, centre national de références maladies autoimmunes systémiques rares, 75013, Paris, France.
Sorbonne universités, UPMC Université Paris 06, Inserm, UMR S 959, immunology immunopathology immunotherapy (I3), 75005, Paris, France; Biotherapy (CIC-BTi) and inflammation-immunopathology-biotherapy department (DHU i2B), hôpital Pitié-Salpêtrière, AP-HP, 75651, Paris, France; AP-HP, groupe hospitalier Pitié-Salpêtrière, department of internal medicine and clinical immunology, centre national de référence maladies autoinflammatoires et amylose inflammatoire, centre national de références maladies autoimmunes systémiques rares, 75013, Paris, France.
Joint Bone Spine. 2019 Nov;86(6):707-713. doi: 10.1016/j.jbspin.2019.01.016. Epub 2019 Feb 4.
Cryoglobulinemia is defined as the persistent presence in serum of abnormal immunoglobulins (Igs) that precipitate at low temperatures and dissolve again upon warming. Cryoglobulins may be composed only of a monoclonal Ig (simple type I cryoglobulinemia), of a monoclonal Ig bound to the constant domain of polyclonal Ig heavy chains (mixed type II cryoglobulinemia), or only of polyclonal Igs (mixed type III cryoglobulinemia). The manifestations of type I cryoglobulinemia are often related to intravascular obstruction, whereas those seen in the mixed cryoglobulinemias often originate in true immune complex-mediated vasculitis. The main clinical manifestations affect the skin (purpura, necrotic ulcers), joints, peripheral nervous system, and kidneys (membranoproliferative glomerulonephritis). Patients with type I cryoglobulinemia should be investigated for hematological malignancies (myeloma and B-cell lymphoma). Hepatitis C is the main diagnosis to consider in patients with mixed cryoglobulinemia, followed by connective tissue disease and B-cell non-Hodgkin's lymphoma. The treatment depends mainly on the cause of the cryoglobulinemia. For instance, hepatitis C virus (HCV) eradication is in order in patients with HCV-associated cryoglobulinemia vasculitis, and the underlying hematological malignancy must be treated in patients with type I cryoglobulinemia.
冷球蛋白血症定义为血清中持续存在异常免疫球蛋白（Ig），这些 Ig 在低温下沉淀，在升温后再次溶解。冷球蛋白可仅由单克隆 Ig 组成（单纯型 I 冷球蛋白血症），由单克隆 Ig 与多克隆 Ig 重链的恒定区结合组成（混合型 II 冷球蛋白血症），或仅由多克隆 Ig 组成（混合型 III 冷球蛋白血症）。I 型冷球蛋白血症的表现通常与血管内阻塞有关，而混合冷球蛋白血症的表现通常源于真正的免疫复合物介导的血管炎。主要的临床表现影响皮肤（紫癜、坏死性溃疡）、关节、周围神经系统和肾脏（膜增殖性肾小球肾炎）。应调查 I 型冷球蛋白血症患者是否存在血液系统恶性肿瘤（骨髓瘤和 B 细胞淋巴瘤）。丙型肝炎是混合冷球蛋白血症患者要考虑的主要诊断，其次是结缔组织疾病和 B 细胞非霍奇金淋巴瘤。治疗主要取决于冷球蛋白血症的病因。例如，丙型肝炎病毒（HCV）根除是 HCV 相关冷球蛋白血症血管炎患者的首要治疗措施，而 I 型冷球蛋白血症患者必须治疗潜在的血液系统恶性肿瘤。
