Laboratory Medicine Program, University Health Network and University of Toronto, Toronto, Canada.
Oslo University Hospital and University of Oslo, Oslo, Norway.
Am J Clin Pathol. 2023 Sep 1;160(3):229-237. doi: 10.1093/ajcp/aqad048.
Primary cold agglutinin disease is a type of autoimmune hemolytic anemia caused by circulating antibodies against I antigen, a carbohydrate expressed on most cells, including red blood cells. The underlying disease has been characterized in recent years as a distinct B-cell lymphoproliferative disease of the bone marrow, occurring mostly in the elderly. The disease has been now been included as a separate entity in the most recent classifications of mature B-cell neoplasms.
A review of the characteristics of cold agglutinin disease is provided, with an emphasis on the pathology features.
A detailed description of the histopathology, immunophenotype, and genetics of cold agglutinin disease is provided and compared to other B-cell lymphoproliferative diseases in the bone marrow with similar features.
Recognition of the pathology features of cold agglutinin disease allows to distinguish it from other diseases, especially lymphoplasmacytic lymphoma and marginal zone lymphoma.
原发性冷凝集素病是一种由针对 I 抗原的循环抗体引起的自身免疫性溶血性贫血,I 抗原是一种存在于大多数细胞(包括红细胞)表面的碳水化合物。近年来,该基础疾病已被确定为一种独特的骨髓 B 细胞淋巴增生性疾病,主要发生在老年人中。该疾病现在已被包含在最新的成熟 B 细胞肿瘤分类中作为一个独立的实体。
对冷凝集素病的特征进行了综述,重点介绍了病理学特征。
详细描述了冷凝集素病的组织病理学、免疫表型和遗传学特征,并与骨髓中具有相似特征的其他 B 细胞淋巴增生性疾病进行了比较。
认识冷凝集素病的病理学特征可将其与其他疾病区分开来,尤其是淋巴浆细胞淋巴瘤和边缘区淋巴瘤。