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唐氏综合征中急性巨核细胞白血病原始细胞的混合表型及短暂异常髓系造血

Mixed phenotype of blasts in acute megakaryocytic leukaemia and transient abnormal myelopoiesis in Down's syndrome.

作者信息

Yumura-Yagi K, Hara J, Kurahashi H, Nishiura T, Kaneyama Y, Osugi Y, Sakata N, Inoue M, Tawa A, Okada S

机构信息

Department of Paediatrics, Osaka University Hospital, Japan.

出版信息

Br J Haematol. 1992 Aug;81(4):520-5. doi: 10.1111/j.1365-2141.1992.tb02985.x.

Abstract

Blasts from eight cases with acute megakaryoblastic leukaemia (AMKL) and seven with transient abnormal myelopoiesis in Down's syndrome (TAM) were investigated to clarify their phenotypic characteristics. CD41 and CD7 were the most frequently expressed in both disorders. CD41 was positive in six TAM and five AMKL cases, and CD7 was positive in five TAM and five AMKL cases, respectively. CD33 was detected in four TAM and five AMKL cases. Other myeloid-lineage associated antigens such as CD13 and CD11b could not be found in TAM but were expressed in five AMKL cases. Interestingly, CD56, a neural adhesion molecule, was expressed in three of four TAM and one of five AMKL cases. Cytoplasmic CD3 antigen was also noted in three of five examined cases. A short-term culture study was conducted on blasts from two TAM cases and five AMKL cases. In two cases in which CD41 was not expressed before culture, the expression of CD41 was enhanced after culture with or without 12-O-tetradecanoyl-phorbol-13-acetate (TPA). The expression of CD7 remarkably was depressed, while that of CD13 was enhanced after culture with TPA. These findings suggest that blasts of TAM and AMKL originate from very immature cells and represent a mixed phenotype. In the present study, distinction of phenotypical differences between blast in TAM and AMKL was not possible.

摘要

对8例急性巨核细胞白血病(AMKL)患者和7例唐氏综合征短暂异常髓系造血(TAM)患者的原始细胞进行了研究,以明确其表型特征。CD41和CD7在这两种疾病中表达最为频繁。CD41在6例TAM和5例AMKL病例中呈阳性,CD7分别在5例TAM和5例AMKL病例中呈阳性。在4例TAM和5例AMKL病例中检测到CD33。其他髓系相关抗原如CD13和CD11b在TAM中未发现,但在5例AMKL病例中表达。有趣的是,神经粘附分子CD56在4例TAM中的3例和5例AMKL中的1例中表达。在所检查的5例病例中的3例中也发现了细胞质CD3抗原。对2例TAM病例和5例AMKL病例的原始细胞进行了短期培养研究。在2例培养前未表达CD41的病例中,无论是否添加12 - O - 十四烷酰佛波醇 - 13 - 乙酸酯(TPA)进行培养,CD41的表达均增强。用TPA培养后,CD7的表达显著降低,而CD13的表达增强。这些发现表明,TAM和AMKL的原始细胞起源于非常不成熟的细胞,并表现出混合表型。在本研究中,无法区分TAM和AMKL原始细胞的表型差异。

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