Patkar Nikhil, Rabade Nikhil, Kadam Pratibha Amare, Mishra Falguni, Muranjan Aditi, Tembhare Prashant, Chaudhary Shruti, Joshi Swapnali, Jain Hasmukh, Dangi Uma, Bagal Bhausaheb, Khattry Navin, Menon Hari, Gujral Sumeet, Sengar Manju, Subramanian P G
Hematopathology Laboratory, Tata Memorial Centre, Parel, Mumbai, Maharashtra, India.
Department of Cancer Cytogenetics, Tata Memorial Centre, Parel, Mumbai, Maharashtra, India.
Indian J Pathol Microbiol. 2017 Jan-Mar;60(1):38-42. doi: 10.4103/0377-4929.200051.
Cytogenetic aberrations as well as presence of IGVH mutations are the underlying reason for clinical heterogeneity in Chronic Lymphocytic Leukemia (CLL). The presence of IGVH mutations as well as the predominant gene usage shows geographical variations. However, there is no study from India addressing immunogenetics of CLL. In a first Indian study we document the immunogenetics of CLL in a large tertiary hospital.
We analyzed IGVH mutation status, VH gene usage, cytogenetic abnormalities using FISH, immunophenotyping data and correlated them with standard clinical variables in 84 patients of CLL.
Advanced Rai stage (Stage 3/4) was seen in 45% of our patients, where as 13q deletion was the commonest clonal cytogenetic abnormality detected in 48.4% of the cases. IGVH unmutated cases (55.2%) showed higher proportion expressing CD38 and CD49d, a preferential usage for VH1 and VH3 families (55.2%), presentation at an advanced Rai stage (52.8%) as well as more frequent presence of p53 deletions. As compared to the IGVH mutated cases greater proportion of IGVH unmutated patients (70%) required treatment. However, there was no significant difference in the time to treatment between mutated and unmutated cases which can be attributed to relatively short median follow up of 10 months.
To summarize, we have seen a higher proportion of IGVH unmutated patients in our cohort (55.2%). The commonly used VH genes in the Indian population are IGVH 2-5, IGVH 1-2 and IGVH 1-69. Longer clinical follow up and a larger cohort is necessary to confirm the prognostic value of IGVH mutation analysis in Indian Patients with CLL.
细胞遗传学异常以及IGHV突变的存在是慢性淋巴细胞白血病(CLL)临床异质性的根本原因。IGHV突变的存在以及主要基因的使用呈现出地域差异。然而,印度尚无关于CLL免疫遗传学的研究。在印度的第一项研究中,我们在一家大型三级医院记录了CLL的免疫遗传学情况。
我们分析了84例CLL患者的IGHV突变状态、VH基因使用情况、采用荧光原位杂交技术检测的细胞遗传学异常以及免疫表型数据,并将它们与标准临床变量进行关联分析。
45%的患者处于Rai晚期(3/4期),13q缺失是最常见的克隆性细胞遗传学异常,在48.4%的病例中被检测到。IGHV未突变的病例(55.2%)显示出较高比例表达CD38和CD49d,优先使用VH1和VH3家族(55.2%),在Rai晚期出现(52.8%)以及更频繁地出现p53缺失。与IGHV突变的病例相比,更大比例的IGHV未突变患者(70%)需要治疗。然而,突变和未突变病例之间的治疗时间没有显著差异,这可能归因于中位随访时间相对较短,仅10个月。
总之,我们的队列中IGHV未突变患者的比例较高(55.2%)。印度人群中常用的VH基因是IGHV 2-5、IGHV 1-2和IGHV 1-69。需要更长时间的临床随访和更大的队列来证实IGHV突变分析对印度CLL患者的预后价值。