MD Anderson Cancer Center, University of Texas, Houston, Texas, USA.
Am J Hematol. 2022 Nov;97 Suppl 2:S26-S34. doi: 10.1002/ajh.26585. Epub 2022 May 12.
Chronic lymphocytic leukemia (CLL) is a common, indolent disease that typically presents with a proliferation of mature, immunologically dysfunctional CD5+ B-cells which preferentially occupy the bone marrow, peripheral blood and lymphoid organs. Immune dysfunction leads to an increase in autoimmune diseases which occur in approximately 10% of patients with CLL. Autoimmune cytopenias are the most common, but other organs may be affected as well. The treatment of these conditions typically depends on the extent of CLL and severity of symptoms, but generally consists of CLL-directed therapies, immunosuppression or both. CLL may also infiltrate extranodal sites in the body. Symptomatic extranodal CLL or extranodal disease which threatens normal organ function is an indication for initiation of CLL-directed therapy. The following review summarizes autoimmune and extranodal complications that can occur in patients with CLL and our suggested approach to their treatment.
慢性淋巴细胞白血病(CLL)是一种常见的、惰性的疾病,通常表现为成熟的、免疫功能失调的 CD5+B 细胞的增殖,这些细胞优先占据骨髓、外周血和淋巴器官。免疫功能失调导致自身免疫性疾病的增加,约 10%的 CLL 患者发生自身免疫性血细胞减少症。自身免疫性血细胞减少症最为常见,但其他器官也可能受到影响。这些情况的治疗通常取决于 CLL 的程度和症状的严重程度,但通常包括 CLL 靶向治疗、免疫抑制或两者兼有。CLL 也可能浸润身体的结外部位。有症状的结外 CLL 或威胁正常器官功能的结外疾病是开始 CLL 靶向治疗的指征。以下综述总结了 CLL 患者可能出现的自身免疫和结外并发症以及我们对其治疗的建议方法。