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巴基斯坦拉合尔儿童医院儿科非霍奇金淋巴瘤的人口统计学特征与结局:单中心经验

Demographics and Outome in Paediatric Non-Hodgkin Lymphoma: Single Centre Experience at The Children Hospital Lahore, Pakistan.

作者信息

Faizan Mahwish, Anwar Saadia, Khan Safia

机构信息

Department of Pediatric Hematology Oncology, he Children Hospital and Institute of Child Health, Lahore.

出版信息

J Coll Physicians Surg Pak. 2018 Jan;28(1):48-51. doi: 10.29271/jcpsp.2018.01.48.

Abstract

OBJECTIVE

To describe the patient demographics and outcome analysis in paediatric non-Hodgkin lymphoma (NHL) patients.

STUDY DESIGN

An observational study.

PLACE AND DURATION OF STUDY

The Hematology/Oncology Unit of The Children's Hospital and Institute of Child Health, Lahore, from January 2012 till December 2014.

METHODOLOGY

Demographics including age, gender, histopathology, stage and outcome data, in biopsy proven NHL patients were analyzed. Burkitts/B Cell and Diffuse Large B Cell lymphoma patients were treated with MCP 842 Protocol while T/B-cell lymphoblastic lymphoma (LL) patients were treated with EURO-LB 02 protocol.

RESULTS

Ninety-one patients were treated during the study period at CHL. Data was insufficient in 18 patients, so they were excluded from the study. Patients included were 73. Males were 53 (72.6%). Thirty-seven (50.7%) were 5-10 years of age, and 22 (30.1%) 10-16 years old. Abdominal mass was the commonest presentation seen in 32 (43.8%), lymphadenopathy in 27 (37%), intussusception in 5 (6.8%), while intestinal obstruction, obstructive uropathy, nasopharyngeal mass, gastric mass, primary bone lymphoma, pericardial effusion, jaw swelling, cheek swelling and paraspinal mass present in one (1%) each. Histopathological subtypes consist of Burkitt's lymphoma (BL) in 32 (43.8%), B cell NHL in 10 (13.7%), lymphoblastic lymphoma (LL) in 26 (35.6%), diffuse large B cell lymphoma (DLBCL) in 2 (2.8%), and anaplastic large cell lymphoma (ALCL) in 1 (1.4%). Sixty-seven (91%) presented in stage III, and six (8.4%) in stage IV. Forty-eight (65.8%) patients had completed treatment and are well to date, 16 (21.9%) died, 5 (6.8%) left against medical advice (LAMA), and 4 (5.5%) patients relapsed.

CONCLUSION

Burkitts lymphoma was the commonest type of NHL seen in this cohort that predominantly presented with an abdominal mass. Children usually presented in advanced stage with delayed diagnosis. Better supportive care can improve the prognosis significantly. Training of pediatricians is equally important along with increasing parental/family knowledge about the disease symptoms so that they can seek early medical care, and earlier diagnosis is possible.

摘要

目的

描述儿童非霍奇金淋巴瘤(NHL)患者的人口统计学特征及预后分析。

研究设计

一项观察性研究。

研究地点及时间

2012年1月至2014年12月,拉合尔儿童医院及儿童健康研究所血液学/肿瘤学部。

方法

对经活检证实的NHL患者的人口统计学特征(包括年龄、性别、组织病理学、分期及预后数据)进行分析。伯基特氏/ B细胞淋巴瘤和弥漫性大B细胞淋巴瘤患者采用MCP 842方案治疗,而T/B细胞淋巴母细胞淋巴瘤(LL)患者采用EURO-LB 02方案治疗。

结果

研究期间,拉合尔儿童医院共治疗了91例患者。18例患者数据不足,因此被排除在研究之外。纳入研究的患者共73例。男性53例(72.6%)。37例(50.7%)年龄在5至10岁之间,22例(30.1%)年龄在10至16岁之间。腹部肿块是最常见的表现,有32例(43.8%);淋巴结病27例(37%);肠套叠5例(6.8%);而肠梗阻、梗阻性尿路病、鼻咽部肿块、胃部肿块、原发性骨淋巴瘤、心包积液、颌部肿胀、面颊肿胀及椎旁肿块各1例(1%)。组织病理学亚型包括伯基特淋巴瘤(BL)32例(43.8%)、B细胞NHL 10例(13.7%)、淋巴母细胞淋巴瘤(LL)26例(35.6%)、弥漫性大B细胞淋巴瘤(DLBCL)2例(2.8%)、间变性大细胞淋巴瘤(ALCL)1例(1.4%)。67例(91%)为Ⅲ期,6例(8.4%)为Ⅳ期。48例(65.8%)患者已完成治疗,目前情况良好;16例(21.9%)死亡;5例(6.8%)违反医嘱出院(LAMA);4例(5.5%)患者复发。

结论

在该队列中,伯基特淋巴瘤是最常见的NHL类型,主要表现为腹部肿块。儿童通常就诊时已处于晚期,诊断延迟。更好的支持性治疗可显著改善预后。对儿科医生的培训以及提高家长/家庭对疾病症状的认识同样重要,以便他们能尽早寻求医疗护理,实现更早诊断。

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