D'Arena Giovanni, Cascavilla Nicola
Hematology Oncology and Bone Marrow Transplantation Unit, National Cancer Institute, IRCCS Fondazione "G. Pascale", Naples, Italy.
Leuk Lymphoma. 2007 Jun;48(6):1072-80. doi: 10.1080/10428190701344923.
The clinical course of patients with B-cell chronic lymphocytic leukemia (CLL) is often made complicated by autoimmune phenomena which mainly target the blood cells. Among them, the autoimmune hemolytic anemia (AIHA) is the most common form. On the other hand, it is believed that CLL is the most common of the known causes of AIHA. The source of any putative autoantibody (bystander nonmalignant cells or tumor cells) is not clear yet. Recently, it has been hypothesized that leukemic B-cells may also act as professional antigen presenting cells (APCs). With respect to the management of CLL-associated AIHA, steroids still represent the first-line treatment option. Intravenous immunoglobulin, immunosuppressive drugs, and splenectomy are also frequently used for steroid-refractory forms. Furthermore, although the case series is still too small, encouraging data is now supporting the use of monoclonal antibodies, in particular anti-CD20 rituximab, in managing this often life-threatening autoimmune complication of CLL.
B 细胞慢性淋巴细胞白血病（CLL）患者的临床病程常因主要针对血细胞的自身免疫现象而变得复杂。其中，自身免疫性溶血性贫血（AIHA）是最常见的形式。另一方面，人们认为 CLL 是已知的 AIHA 最常见病因。任何假定的自身抗体（旁观者非恶性细胞或肿瘤细胞）的来源尚不清楚。最近，有人提出白血病 B 细胞也可能作为专职抗原呈递细胞（APC）发挥作用。关于 CLL 相关 AIHA 的治疗，类固醇仍然是一线治疗选择。静脉注射免疫球蛋白、免疫抑制药物和脾切除术也常用于类固醇难治性病例。此外，尽管病例系列仍然太小，但目前令人鼓舞的数据支持使用单克隆抗体，特别是抗 CD20 利妥昔单抗，来治疗 CLL 这种常危及生命的自身免疫并发症。
