The t(6;9)(p22;q34) in myeloid neoplasms: a retrospective study of 16 cases

Monika Gupta , J. Ashok Kumar , Usha Sitaram , S. Neeraj , A. Nancy , Poonkuzhali Balasubramanian , Aby Abraham , Vikram Mathews , Auro Viswabandya , Biju George , Mammen Chandy , Alok Srivastava , Vivi M. Srivastava
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引用次数: 29

Abstract

Among patients with acute myeloid leukemia (AML), the t(6;9) (p22;q34) is a rare but defined subset with a poor prognosis. We report 16 patients with the t(6;9), of whom 13 had AML, 2 had myelodysplastic syndrome (MDS), and 1 had chronic myeloid leukemia in myeloid blast crisis (CML-BC). All except for one were evaluated at diagnosis. The median age was 34.5 (range: 7–62 years), with 12 adults and 12 males. Trilineage dysplasia was present in 13 (81%). Marrow basophilia was seen in only two patients, one of whom had CML-BC. HLA-DR was positive in all 12 patients assessed, CD33 in 11, CD13 in 10, and CD34 in seven. Four patients had one other abnormality apart from the t(6;9). These were the t(9;22) in the patient with CML and deletion 9q, addition 13q, and an isochromosome 8q in the other three patients. There were no complex karyotypes. Fms-related tyrosine kinase 3—internal tandem duplication (FLT3-ITD) mutations were seen in seven of 13 patients. Follow-up details were available for six patients. Three received palliative care, and follow-up details were not available for the other seven. The response to chemotherapy was poor in the remaining patients. The only patients who survived were three out of the four who had allogeneic hematopoietic stem cell transplantation (HSCT).

髓系肿瘤的t(6;9)(p22;q34): 16例回顾性研究
在急性髓性白血病(AML)患者中,t(6;9) (p22;q34)是一个罕见但明确的亚群,预后较差。我们报告了16例t(6;9)患者,其中13例患有AML, 2例患有骨髓增生异常综合征(MDS), 1例患有慢性髓细胞白血病髓细胞危像(CML-BC)。除一人外,其余均在诊断时进行评估。年龄中位数为34.5岁(范围7-62岁),成人12人,男性12人。13例(81%)出现三岁发育不良。只有2例患者出现骨髓嗜碱性,其中1例为CML-BC。所有12例患者HLA-DR均呈阳性,其中11例为CD33, 10例为CD13, 7例为CD34。4例患者除t外还有另外1个异常(6;9)。这些是CML患者的t(9;22),缺失9q,增加13q,其他三名患者的同工染色体8q。没有复杂的核型。13例患者中有7例出现fms相关酪氨酸激酶3-内串联重复(FLT3-ITD)突变。有6例患者的随访细节。其中3人接受了姑息治疗,其他7人的随访细节不详。其余患者对化疗的反应较差。4名接受同种异体造血干细胞移植(HSCT)的患者中只有3名存活下来。
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