Stem Cell Transplant and Cellular Therapies, University of Iowa Hospitals and Clinics, Iowa City, IA, USA.
J Oncol Pharm Pract. 2023 Oct;29(7):1785-1788. doi: 10.1177/10781552231185153. Epub 2023 Jul 4.
Autologous hematopoietic stem cell transplant is an important treatment modality used to achieve long-term remission in people with multiple myeloma. Complications include chemotherapy-related toxicity or infection. Rarely, clinical features consistent with autologous graft-versus-host disease, otherwise known as auto-aggression syndrome, is possible. Auto-aggression syndrome appears more commonly in patients with multiple myeloma, hypothesized to be a result of underlying immune dysregulation, conditioning chemotherapy, or treatment with immunomodulating agents.
A 66-year-old female with multiple myeloma underwent an autologous stem cell transplant with melphalan conditioning chemotherapy followed by maintenance therapy with lenalidomide. Transplant was complicated by engraftment syndrome versus auto-aggression syndrome. After lenalidomide maintenance therapy initiation, she required hospitalization for auto-aggression syndrome.
Auto-aggression syndrome with gastrointestinal, hepatic, and dermatologic involvement as demonstrated by skin punch biopsy, elevated reg3α, ST2, elafin, eosinophilia, transaminitis, and persistent diarrhea beyond the engraftment period were noted. Topical and systemic steroids with a prolonged taper resulted in symptom resolution.
Acute graft-versus-host disease is a complication once considered unique to allogeneic stem cell transplant recipients, but a similar syndrome termed "auto-aggression syndrome" may be seen following autologous transplant. Auto-aggression syndrome should be suspected when complications extend beyond the normal engraftment syndrome period following autologous transplant, particularly in people with multiple myeloma, and/or those who have received prior immunomodulating therapy. There should be a low threshold for obtaining biopsies in the setting of suspected auto-aggression syndrome. Early recognition and prompt initiation of corticosteroids with prolonged tapers may prevent auto-aggression syndrome relapse and readmissions.
自体造血干细胞移植是多发性骨髓瘤患者实现长期缓解的重要治疗方式。其并发症包括化疗相关毒性或感染。罕见情况下，可能会出现与自体移植物抗宿主病一致的临床特征，即所谓的自体攻击综合征。自体攻击综合征在多发性骨髓瘤患者中更为常见，推测是由于潜在免疫失调、预处理化疗或免疫调节药物治疗所致。
一名 66 岁女性患有多发性骨髓瘤，接受了自体干细胞移植，并用马法兰进行预处理化疗，随后接受来那度胺维持治疗。移植后并发移植物抗宿主病与自体攻击综合征。开始来那度胺维持治疗后，因自体攻击综合征住院。
出现胃肠道、肝脏和皮肤受累的自体攻击综合征，表现为皮肤打孔活检显示升高的 reg3α、ST2、elafin、嗜酸性粒细胞增多、转氨酶升高和移植后持续腹泻。给予皮质类固醇的局部和全身治疗，并进行了长时间的减量，症状得到缓解。
急性移植物抗宿主病曾被认为是异基因干细胞移植受者所特有的并发症，但在自体移植后也可能出现类似的综合征，称为“自体攻击综合征”。在自体移植后，当并发症超出正常移植物抗宿主病期时，尤其是在多发性骨髓瘤患者和/或接受过免疫调节治疗的患者中，应怀疑自体攻击综合征。在疑似自体攻击综合征的情况下，应降低获取活检的门槛。早期识别并及时开始长期皮质类固醇减量治疗可能预防自体攻击综合征复发和再入院。
