Gertz Morie A
Mayo Clinic, Two Hundred South West 1st Street, Rochester, MN 55905, USA. Electronic address: https://twitter.com/moriegertz.
Hematol Oncol Clin North Am. 2022 Apr;36(2):341-352. doi: 10.1016/j.hoc.2021.11.001. Epub 2022 Mar 11.
Cold agglutinin disease represents a form of immune-mediated hemolytic anemia whereby an IgM protein either monoclonal or polyclonal deposits complement on the surface of the red blood cell. Once complement is deposited, the 3rd component of complement is recognized by receptors in the mononuclear phagocyte system resulting in spherocytic extravascular hemolysis. This results in a Coombs positive hemolytic anemia with the peripheral blood film showing agglutination. In many instances, the source is a clonal population of lymphoplasmacytic cells in the bone marrow producing a monoclonal IgM protein. Traditional and emerging therapies directed against the production of the IgM may have a positive effect on hemolytic anemia. Success in the management of cold agglutinin disease with rituximab, fludarabine, bortezomib, and bendamustine has all been reported. Recent studies have demonstrated that the blockade of complement with sutimlimab can stop the hemolysis without the use of systemic chemotherapy.
冷凝集素病是一种免疫介导的溶血性贫血，其中IgM蛋白（单克隆或多克隆）在红细胞表面沉积补体。一旦补体沉积，补体的第三成分被单核吞噬细胞系统中的受体识别，导致球形红细胞血管外溶血。这会导致抗人球蛋白试验阳性的溶血性贫血，外周血涂片显示凝集。在许多情况下，病因是骨髓中产生单克隆IgM蛋白的淋巴浆细胞克隆群体。针对IgM产生的传统和新兴疗法可能对溶血性贫血有积极作用。已报道使用利妥昔单抗、氟达拉滨、硼替佐米和苯达莫司汀治疗冷凝集素病取得成功。最近的研究表明，用苏替利单抗阻断补体可以在不使用全身化疗的情况下停止溶血。
