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急性双系白血病:一种预后不良的罕见疾病。

Acute bilineal leukemia: a rare disease with poor outcome.

作者信息

Weir E G, Ali Ansari-Lari M, Batista D A S, Griffin C A, Fuller S, Smith B D, Borowitz M J

机构信息

Department of Pathology, Johns Hopkins Medical Institutions, Baltimore, MD 21231, USA.

出版信息

Leukemia. 2007 Nov;21(11):2264-70. doi: 10.1038/sj.leu.2404848. Epub 2007 Jul 5.

Abstract

Most cases of acute leukemia can be assigned to the myeloid, B or T lineage. In a few cases, definitive assignment cannot be achieved because blasts express antigens of more than one lineage. A subset of these, referred to as acute bilineal leukemias (aBLLs), is characterized by the presence of more than one population of blasts, each comprising a single lineage. We identified 19 cases of aBLL, including 10 mixed T and myeloid (T-My) and nine mixed B and myeloid (B-My); no mixed B and T cases were identified. Cytogenetic data were available for 16 patients. Three of seven patients with B-My had a t(9;22)(q34q11.2), two had 11q23 translocations and one had del(9). Two of nine patients with T-My had 2p13 translocations; five had other unrelated abnormalities. Of 16 patients with outcome data, only six achieved complete remission and only two remain free of disease 2.5 and 4.5 years after chemotherapy or stem cell transplantation. aBLL is a rare disease that combines B or T and myeloid blasts. Cytogenetic abnormalities of t(9;22) and 11q23 are common in, and may be restricted to, B-My cases, while T-My cases have frequent but generally non-recurring abnormalities. Both types of aBLL are associated with poor outcome.

摘要

大多数急性白血病病例可归为髓系、B 系或 T 系。在少数情况下,由于原始细胞表达不止一种系的抗原,无法进行明确归类。其中一部分被称为急性双系白血病(aBLLs),其特征是存在不止一种原始细胞群,每个群均由单一系组成。我们鉴定出 19 例 aBLL,包括 10 例混合 T 系和髓系(T-My)以及 9 例混合 B 系和髓系(B-My);未鉴定出混合 B 系和 T 系病例。16 例患者有细胞遗传学数据。7 例 B-My 患者中,3 例有 t(9;22)(q34q11.2),2 例有 11q23 易位,1 例有 9 号染色体缺失。9 例 T-My 患者中,2 例有 2p13 易位;5 例有其他不相关异常。在有结局数据的 16 例患者中,仅 6 例达到完全缓解,化疗或干细胞移植后 2.5 年和 4.5 年时仅有 2 例仍无病生存。aBLL 是一种罕见疾病,合并了 B 系或 T 系以及髓系原始细胞。t(9;22)和 11q23 的细胞遗传学异常在 B-My 病例中常见,且可能仅限于此类病例,而 T-My 病例有常见但一般不复发的异常。两种类型的 aBLL 均与不良结局相关。

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