Division of Hematology, Department of Medicine, Johns Hopkins University School of Medicine, 1830 East Monument Street, Suite 7300, Baltimore, MD 21205, USA.
Division of Hematology, Johns Hopkins University School of Medicine, 720 Rutland Ave, Ross Building Rm 1025, Baltimore, MD 21205, USA.
Hematol Oncol Clin North Am. 2022 Apr;36(2):325-339. doi: 10.1016/j.hoc.2021.11.005. Epub 2022 Mar 11.
Warm autoimmune hemolytic anemia (wAIHA) is an uncommon and heterogeneous disorder caused by autoantibodies to RBC antigens. Initial evaluation should involve the DAT, with wAIHA typically IgG positive with or without C3 positivity, and a search for underlying conditions associated with secondary wAIHA, which comprise 50% of cases. First-line therapy involves glucocorticoids, increasingly with rituximab, though a chronic relapsing course is typical. While splenectomy and a number of immunosuppressive therapies have been used in the setting of relapsed and refractory disease, the optimal choice and sequence of therapies is unknown, and clinical trials should be offered when available. Newer investigational targets include spleen tyrosine kinase inhibitors, monoclonal antibodies targeting CD38, Bruton's tyrosine kinase inhibitors, complement inhibitors, and antibodies against neonatal Fc receptors.
温抗体型自身免疫性溶血性贫血(wAIHA)是一种由针对 RBC 抗原的自身抗体引起的不常见且异质性的疾病。初始评估应包括 DAT,wAIHA 通常 IgG 阳性,伴有或不伴有 C3 阳性,并寻找与继发性 wAIHA 相关的潜在情况,这占病例的 50%。一线治疗包括糖皮质激素,越来越多地与利妥昔单抗联合使用,但典型的是慢性复发性病程。虽然脾切除术和许多免疫抑制疗法已用于复发和难治性疾病,但最佳选择和治疗顺序尚不清楚,应在有条件时提供临床试验。新的研究靶点包括脾酪氨酸激酶抑制剂、针对 CD38 的单克隆抗体、布鲁顿酪氨酸激酶抑制剂、补体抑制剂和针对新生儿 Fc 受体的抗体。