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伴有inv(3)(q21q26.2)或t(3;3)(q21;q26.2)的急性髓系白血病:临床和生物学特征以及与其他伴有涉及3号染色体长臂细胞遗传学异常的急性髓系白血病的比较

Acute myeloid leukemia with inv(3)(q21q26.2) or t(3;3)(q21;q26.2): Clinical and biological features and comparison with other acute myeloid leukemias with cytogenetic aberrations involving long arm of chromosome 3.

作者信息

Raya Jose Maria, Martín-Santos Taida, Luño Elisa, Sanzo Carmen, Perez-Sirvent Maria Luz, Such Esperanza, Navarro José Tomás, Millá Fuensanta, Alonso Esther, Domingo Alicia, Rozman María, Díaz-Beva Marina, Batlle Ana, González-de-Villambrosia Sonia, Tuset Esperanza, Vallespí Teresa, Ortega Margarita, Bermejo Alfredo, Martín-Ramos Marisa, Peri Valeria, Solé Francesc, Florensa Lourdes

机构信息

a Department of Hematology Hospital Universitario de Canarias , Ofra s/n, 38320 La Laguna, Spain.

出版信息

Hematology. 2015 Sep;20(8):435-441. doi: 10.1179/1607845415Y.0000000003. Epub 2015 Feb 13.

Abstract

OBJECTIVES

To compare, from a biological and clinical perspective, a significant group of patients with AML with inv(3)(q21q26.2) or t(3;3)(q21;q26.2) with another group of AML carrying different abnormalities of 3q at q21 or q26, the latter named as the AML abn(3q) group.

METHODS

We developed a national survey with the participation of 13 Spanish hospitals, and retrospectively reviewed (from 1990 to 2010) these subtypes of AML. Fifty-five patients were collected: 35 with AML inv(3)/t(3;3) and 20 with AML abn(3q). A data collecting page that included main features at diagnosis, therapeutic approach and response, and survival variables, was distributed and completed.

RESULTS

We did not find significant differences in sex, age, history of myelodysplastic syndrome or chemo-/radiotherapy, clinical presentation, WBC and platelet counts, hemoglobin level, blasts immunophenotype, serum lactatedehydrogenase, peripheral blood and bone marrow cellular dysplasia, and bone marrow biopsy findings. Although the association with monosomy 7 was significantly more frequent in AML inv(3)/t(3;3), this did not seem to influence outcome. The lack of response to the different modalities of treatment and the aggressive course of the disease were the standard in both cohorts of patients.

DISCUSSION

Although not yet recognized by the World Health Organization classification, our results are in agreement with the findings of other authors, who include both subsets of AML together in the same group of adverse prognosis.

CONCLUSION

In an attempt to simplify and bound entities with similar genetic background and clinical behavior, it would be desirable to bring together both subgroups of AML in a single section.

摘要

目的

从生物学和临床角度比较一组伴有inv(3)(q21q26.2)或t(3;3)(q21;q26.2)的急性髓系白血病(AML)患者与另一组伴有3号染色体长臂(3q)在q21或q26处有不同异常的AML患者(后者命名为AML abn(3q)组)。

方法

我们开展了一项有13家西班牙医院参与的全国性调查,并对这些AML亚型进行回顾性分析(时间跨度为1990年至2010年)。共收集了55例患者:35例为AML inv(3)/t(3;3)患者,20例为AML abn(3q)患者。发放并填写了一份数据收集表,内容包括诊断时的主要特征、治疗方法及反应以及生存变量。

结果

我们未发现两组患者在性别、年龄、骨髓增生异常综合征病史或化疗/放疗史、临床表现、白细胞和血小板计数、血红蛋白水平、原始细胞免疫表型、血清乳酸脱氢酶、外周血和骨髓细胞发育异常以及骨髓活检结果方面存在显著差异。虽然AML inv(3)/t(3;3)与7号染色体单体的关联更为常见,但这似乎并未影响预后。两组患者对不同治疗方式均缺乏反应且疾病进展迅速。

讨论

尽管世界卫生组织分类尚未认可,但我们的结果与其他作者的发现一致,他们将这两个AML亚组归为同一不良预后组。

结论

为了简化并整合具有相似遗传背景和临床行为的实体,将AML的这两个亚组合并在一个单独的章节中是可取的。

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