Derwich Katarzyna, Sedek Lukasz, Meyer Claus, Pieczonka Anna, Dawidowska Małgorzata, Gaworczyk Anna, Wachowiak Jacek, Konatkowska Benigna, Witt Michał, Marschalek Rolf, Szczepański Tomasz
Department of Pediatric Hematology, Oncology and Haematopoietic Stem Cell Transplantation, University of Medical Sciences, Szpitalna 27/33, 60-572 Poznań, Poland.
Leuk Res. 2009 Jul;33(7):1005-8. doi: 10.1016/j.leukres.2009.02.007. Epub 2009 Mar 16.
Most cases of acute leukemia can be assigned to the myeloid, B or T lineage. There are rare cases of acute leukemia, which cannot be clearly classified, because either blasts express antigens of more than one lineage (acute biphenotypic leukemias) or distinct blast populations of two lineages co-exist (acute bilineal leukemias, aBLL). We present a 10-month-old infant with de novo aBLL, characterized by blasts of monocytic and B-cell precursor lineages. All leukemic cells harbored identical complex MLL gene rearrangement. Despite poor initial response, both to acute lymphoblastic leukemia (ALL) induction treatment and acute myeloid leukemia induction blocks, the child reached complete clinical remission with minimal residual disease negative status and was transplanted. Unfortunately, 16 months from HSCT the patient experienced BM relapse with all blasts characterized by pro-B-ALL immunophenotype. This case report illustrates that aBLL is a very aggressive type of acute leukemia that should be individually treated and monitored, particularly in children less than 1 year of age.
大多数急性白血病病例可归类为髓系、B 系或 T 系。有少数急性白血病病例无法明确分类，原因要么是原始细胞表达一种以上系的抗原（急性双表型白血病），要么是两个系的不同原始细胞群共存（急性双系白血病，aBLL）。我们报告了一名 10 个月大的初发 aBLL 婴儿，其特征为单核细胞和 B 细胞前体系的原始细胞。所有白血病细胞均存在相同的复杂 MLL 基因重排。尽管对急性淋巴细胞白血病（ALL）诱导治疗和急性髓系白血病诱导方案的初始反应均不佳，但该患儿达到了完全临床缓解，微小残留病呈阴性状态并接受了移植。不幸的是，异基因造血干细胞移植（HSCT）后 16 个月，患者出现骨髓复发，所有原始细胞均具有前体 B-ALL 免疫表型。本病例报告表明，aBLL 是一种侵袭性很强的急性白血病类型，应进行个体化治疗和监测，尤其是对于 1 岁以下的儿童。
