Villeval J L, Cramer P, Lemoine F, Henri A, Bettaieb A, Bernaudin F, Beuzard Y, Berger R, Flandrin G, Breton-Gorius J
Blood. 1986 Nov;68(5):1167-74.
Nine cases of early erythroblastic leukemia, unidentified by usual criteria, have been diagnosed using a panel of antibodies. Three cases arose in patients with Down's syndrome, one in a patient with therapy-related leukemia, and four patients were in blast crisis of chronic myeloid leukemia; only one case arose de novo. Blast cells could be assigned to two main stages of erythroid differentiation: presence of all erythroid-specific proteins in two patients, a phenotype corresponding to an immature erythroblast; absence of the erythroid markers such as glycophorin A and spectrin in the presence of carbonic anhydrase isoenzyme I, ABH group antigens, and the antigen defined by FA6 152 monoclonal antibody in six patients, a phenotype related to a late erythroid progenitor (CFU-E). One patient had an intermediate phenotype. All patients except one demonstrated a megakaryocytic component. In three patients, chromosomal abnormalities were present, detected both in blasts and in erythroid colonies. In conclusion, these findings indicate that most "cryptic erythroleukemias" are blocked at a "CFU-E-like" stage of differentiation, it may be a frequent event in Down's syndrome and chronic myeloid leukemia, and these erythroleukemias are phenotypically heterogeneous.
使用一组抗体诊断出9例常规标准无法识别的早期成红细胞白血病。3例发生在唐氏综合征患者中,1例发生在治疗相关白血病患者中,4例处于慢性粒细胞白血病急变期;仅1例为原发性。原始细胞可分为红系分化的两个主要阶段:2例患者存在所有红系特异性蛋白,其表型对应未成熟成红细胞;6例患者在存在碳酸酐酶同工酶I、ABH血型抗原以及FA6 152单克隆抗体所定义的抗原时,缺乏血型糖蛋白A和血影蛋白等红系标志物,其表型与晚期红系祖细胞(CFU-E)相关。1例患者具有中间表型。除1例患者外,所有患者均显示有巨核细胞成分。3例患者存在染色体异常,在原始细胞和红系集落中均检测到。总之,这些发现表明,大多数“隐匿性红白血病”在“CFU-E样”分化阶段受阻,这在唐氏综合征和慢性粒细胞白血病中可能是常见现象,并且这些红白血病在表型上具有异质性。