Johansen Silje, Kollsete Gjelberg Hilde, Ahmed Aymen Bushra, Bruserud Øystein, Reikvam Håkon
Section of Hematology, Department of Medicine, Haukeland University Hospital, 5021 Bergen, Norway.
Department of Pathology, Haukeland University Hospital, 5021 Bergen, Norway.
Case Rep Oncol Med. 2018 Feb 28;2018:7697283. doi: 10.1155/2018/7697283. eCollection 2018.
Myeloid sarcoma is an extramedullary (EM) manifestation (i.e., manifestation outside the bone marrow) of acute myeloid leukemia (AML); it is assumed to be relatively uncommon and can be the only manifestation of leukemia relapse after allogenic stem cell transplantation (allo-SCT). An EM sarcoma can manifest in any part of the body, although preferentially manifesting in immunological sanctuary sites as a single or multiple tumors. The development of myeloid sarcoma after allo-SCT is associated with certain cytogenetic abnormalities, developing of graft versus host disease (GVHD), and treatment with donor lymphocytes infusion (DLI). It is believed that posttransplant myeloid sarcomas develop because the EM sites evade immune surveillance. We present two patients with EM myeloid sarcoma in the breast and epipharynx, respectively, as the only manifestation of leukemia relapse. Both patients were treated with a combination of local and systemic therapy, with successfully longtime disease-free survival. Based on these two case reports, we give an updated review of the literature and discuss the pathogenesis, diagnosis, and treatment of EM sarcoma as the only manifestation of AML relapse after allo-SCT. There are no standard guidelines for the treatment of myeloid sarcomas in allotransplant recipients. In our opinion, the treatment of these patients needs to be individualized and should include local treatment (i.e., radiotherapy) combined with systemic therapy (i.e., chemotherapy, immunotherapy, DLI, or retransplantation). The treatment has to consider both the need for sufficient antileukemic efficiency versus the risk of severe complications due to cumulative toxicity.
髓系肉瘤是急性髓系白血病(AML)的一种髓外(EM)表现(即骨髓外的表现);据推测其相对不常见,并且可能是异基因干细胞移植(allo - SCT)后白血病复发的唯一表现。EM肉瘤可在身体的任何部位出现,尽管更倾向于在免疫庇护部位以单个或多个肿瘤的形式出现。allo - SCT后髓系肉瘤的发生与某些细胞遗传学异常、移植物抗宿主病(GVHD)的发生以及供体淋巴细胞输注(DLI)治疗有关。据信移植后髓系肉瘤的发生是因为EM部位逃避了免疫监视。我们分别报告了两名患者,其乳腺和会咽部出现EM髓系肉瘤,作为白血病复发的唯一表现。两名患者均接受了局部和全身治疗的联合方案,均成功实现了长期无病生存。基于这两例病例报告,我们对文献进行了更新回顾,并讨论了作为allo - SCT后AML复发唯一表现的EM肉瘤的发病机制、诊断和治疗。对于同种异体移植受者中髓系肉瘤的治疗,目前尚无标准指南。我们认为,这些患者的治疗需要个体化,应包括局部治疗(即放疗)与全身治疗(即化疗、免疫治疗、DLI或再次移植)相结合。治疗必须兼顾足够的抗白血病疗效需求与因累积毒性导致严重并发症的风险。