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真性红细胞增多症后急性白血病的免疫表型特征

Immunophenotypic characterisation of acute leukaemia after polycythemia vera.

作者信息

Hernández J M, Orfao A, González M, Cuesta B, López-Berges M C, Cañizo M C, Ciudad J, San Miguel J F

机构信息

Servico de Hematología, Hospital Clínico Universitario, Salamanca, Spain.

出版信息

J Clin Pathol. 1993 Jul;46(7):668-71. doi: 10.1136/jcp.46.7.668.

Abstract

AIMS

To analyze the immunophenotype of blast cells in patients with acute leukaemia after polycythemia vera, together with the most relevant clinical and haematological disease characteristics.

METHODS

The immunophenotype was analysed in nine patients by immunofluorescence flow cytometry using a panel of 15 monoclonal antibodies. The DNA content of blast cells was determined using Vindelov's technique.

RESULTS

The most relevant clinical and haematological disease characteristics included: the presence of enlarged spleen and liver by 56% and 67%, respectively; a moderate degree of leucocytosis with thrombocytopenia while haemoglobin was normal in 50% of patients. All patients received alkylating agents or hydroxyurea, or both. Interestingly, the chronic phase in patients receiving this latter drug was shorter. All cases showed a myeloid phenotype, four of them reactive only to early myeloid antigens (CD13/33); in the remaining cases the blast cells displayed granulomonocytic (CD14+, CD15+), erythroid (CD71 ), or megakaryocytic (CD61+, CD41+) markers. Coexpression of lymphoid related antigens (CD7, TdT, or CD19) was also detected. The morphological assessment of blast cells agreed with the immunophenotyping in five out of the nine cases. Blast cells from all six patients analysed displayed a diploid DNA content and the proportion of S-phase cells ranged from 0.4% to 4%.

CONCLUSIONS

These findings suggest a pluripotential stem cell with myeloid commitment as the target cell of acute leukaemia after polycythemia vera.

摘要

目的

分析真性红细胞增多症后急性白血病患者原始细胞的免疫表型,以及最相关的临床和血液学疾病特征。

方法

使用一组15种单克隆抗体,通过免疫荧光流式细胞术对9例患者的免疫表型进行分析。采用温德洛夫技术测定原始细胞的DNA含量。

结果

最相关的临床和血液学疾病特征包括:分别有56%和67%的患者出现脾脏和肝脏肿大;50%的患者白细胞中度增多伴血小板减少,而血红蛋白正常。所有患者均接受了烷化剂或羟基脲,或两者都接受。有趣的是,接受后一种药物治疗的患者慢性期较短。所有病例均表现为髓系表型,其中4例仅对早期髓系抗原(CD13/33)有反应;其余病例中原始细胞显示粒单核细胞(CD14+、CD15+)、红系(CD71)或巨核系(CD61+、CD41+)标记。还检测到淋巴相关抗原(CD7、TdT或CD19)的共表达。9例病例中有5例原始细胞的形态学评估与免疫表型分析结果一致。分析的所有6例患者的原始细胞均显示二倍体DNA含量,S期细胞比例为0.4%至4%。

结论

这些发现提示真性红细胞增多症后急性白血病的靶细胞是具有髓系定向的多能干细胞。

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Cell lineage heterogeneity in blast crisis of chronic myeloid leukaemia.
Br J Haematol. 1985 Mar;59(3):395-409. doi: 10.1111/j.1365-2141.1985.tb07326.x.
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Surface marker analysis in acute myeloid leukaemia and correlation with FAB classification.
Br J Haematol. 1986 Nov;64(3):547-60. doi: 10.1111/j.1365-2141.1986.tb02211.x.

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