儿童获得性再生障碍性贫血中的阵发性睡眠性血红蛋白尿表型细胞和白细胞亚群端粒长度
Paroxysmal nocturnal haemoglobinuria phenotype cells and leucocyte subset telomere length in childhood acquired aplastic anaemia.
作者信息
Tutelman Perri R, Aubert Geraldine, Milner Ruth A, Dalal Bakul I, Schultz Kirk R, Deyell Rebecca J
机构信息
Division of Pediatric Hematology/Oncology/Bone Marrow Transplantation, British Columbia Children's Hospital, University of British Columbia, Vancouver, BC, Canada.
出版信息
Br J Haematol. 2014 Mar;164(5):717-21. doi: 10.1111/bjh.12656. Epub 2013 Nov 14.
The significance of paroxysmal nocturnal haemoglobinuria (PNH(pos) ) cells and leucocyte subset telomere lengths in paediatric aplastic anaemia (AA) is unknown. Among 22 children receiving immunosuppressive therapy (IST) for AA, 73% (16/22) were PNH(pos) , of whom 94% achieved at least a partial response (PR) to IST; 11/16 (69%) achieved complete response (CR). Only 2/6 (33%) PNH(neg) patients achieved PR. PNH(pos) patients were less likely to fail IST compared to PNH(neg) patients (odds ratio 0·033; 95% confidence interval 0·002-0·468; P = 0·012). Children with AA had short granulocyte (P = 7·8 × 10(-9) ), natural killer cell (P = 6·0 × 10(-4) ), naïve T lymphocyte (P = 0·002) and B lymphocyte (P = 0·005) telomeres compared to age-matched normative data.
阵发性夜间血红蛋白尿（PNH阳性）细胞和白细胞亚群端粒长度在儿童再生障碍性贫血（AA）中的意义尚不清楚。在22名接受免疫抑制治疗（IST）的AA患儿中，73%（16/22）为PNH阳性，其中94%对IST至少有部分缓解（PR）；11/16（69%）达到完全缓解（CR）。只有2/6（33%）的PNH阴性患者达到PR。与PNH阴性患者相比，PNH阳性患者IST治疗失败的可能性较小（优势比0·033；95%置信区间0·002 - 0·468；P = 0·012）。与年龄匹配的正常数据相比，AA患儿的粒细胞（P = 7·8 × 10⁻⁹）、自然杀伤细胞（P = 6·0 × 10⁻⁴）、初始T淋巴细胞（P = 0·002）和B淋巴细胞（P = 0·005）端粒较短。
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