Abu-Arja Rolla F, Dargart Jamie L, Bajwa Rajinder P S, Kahwash Samir B, Auletta Jeffery J, Rangarajan Hemalatha G
Department of Pediatric Hematology, Oncology and Bone Marrow Transplant, Nationwide Children's Hospital, Columbus, Ohio.
Department of Pediatrics, The Ohio State University, Columbus, Ohio.
Pediatr Transplant. 2018 Dec;22(8):e13291. doi: 10.1111/petr.13291. Epub 2018 Sep 15.
AML with the RAM immunophenotype is associated with extremely poor prognosis. We report a rare case of monozygotic twins presenting simultaneously at the age of 2 years with RAM AML. Each twin underwent a myeloablative 7/10 unrelated umbilical cord blood transplant. Pretransplant Twin A's bone marrow was negative for MRD by flow cytometry (<0.01%) unlike Twin B's bone marrow (0.07%). Twin A is alive in remission 3 years from transplant. Twin B developed primary graft failure, but subsequently rescued with a haploidentical stem cell transplant. However, she relapsed and died 13 months from diagnosis. The twins' clinical courses demonstrate that upfront intensive chemotherapy to achieve negative MRD, followed by allogeneic hematopoietic stem cell transplant as postremission intensification strategy, should be considered in this high-risk AML.
具有RAM免疫表型的急性髓系白血病(AML)预后极差。我们报告了一例罕见的同卵双胞胎病例,这对双胞胎在2岁时同时被诊断为RAM AML。每个双胞胎均接受了清髓性7/10非亲缘脐血移植。移植前,流式细胞术检测显示双胞胎A的骨髓微小残留病(MRD)为阴性(<0.01%),而双胞胎B的骨髓MRD为0.07%。双胞胎A移植后3年仍处于缓解期存活。双胞胎B发生了原发性移植物衰竭,但随后通过单倍体干细胞移植得到挽救。然而,她在诊断后13个月复发并死亡。这对双胞胎的临床病程表明,对于这种高危AML,应考虑采用前期强化化疗以实现MRD阴性,随后进行异基因造血干细胞移植作为缓解后强化策略。