儿童急性淋巴细胞白血病合并噬血细胞综合征14例临床分析

Analysis of 14 children with acute lymphocytic leukemia associated haemophagocytic lymphohistiocytosis

目的探讨儿童急性淋巴细胞白血病(ALL)合并噬血细胞综合征(HPS)的临床特征、治疗方法及预后。方法收集郑州大学第一附属医院儿童医院近6 a确诊的14例ALL合并HPS患儿,回顾性分析其临床特征、实验室检查、治疗与转归。结果 (1)14例患儿中,急性B淋巴细胞白血病(ALL-B)11例,急性T淋巴细胞白血病(ALL-T)3例。(2)临床表现:发热100. 0%(24/24)、脾肿大42. 9%(6/14)、肝肿大42. 9%(6/14)、淋巴结肿大28. 6%(4/14)、黄疸7. 1%(1/14)、浆膜腔积液35. 7%(5/14)、皮肤黏膜出血14. 3%(2/14)、呼吸系统症状92. 9%(13/14)、神经系统症状7. 1%(1/14);实验室检查:全血细胞减少100. 0%(14/14)、血清铁蛋白升高92. 9%(13/14)、甘油三酯升高21. 4%(3/14)、纤维蛋白原降低35. 7%(5/14)、骨髓吞噬现象71. 4%(10/14)、乳酸脱氢酶水平升高64. 3%(9/14)、肝功能异常71. 4%(10/14)、C反应蛋白水平升高78. 6%(11/14)。(3)给予12例患儿HLH-2004方案治疗,2例放弃治疗。结论儿童ALL合并HPS临床表现多样,常伴严重感染、多脏器功能损伤,治疗效果极差,死亡率极高。

Objective To investigate the clinical characteristics and prognosis of acute lymphocytic leukemia associated haemophagocytic lymphohistiocytosis in children. Methods A retrospective study was conducted to analyze the pathogenesis, clinical characteristics, laboratory findings and outcomes of 14 patients with acute lymphocytic leukemia associated haemophagocytic lymphohistiocytosis. Results （1）Eleven cases were acute B lymphocytic leukemia（ B - ALL） , and 3 cases were acute T lympho cytic leukemia（T - ALL）. （2）Clinical symptoms： fever 100. 0% （14/14） , splenomegaly 42. 9% （6/14）, hepatomegaly 42. 9% （6/14）, lymphadenopathy 28.6% （4/14）, jaundice 7. 1% （ 1/14）, dropsy of serous cavity 35.7% （5/14）, skin purpura 14. 3 % （2/14）, respiratory symptoms 92. 9% （ 13/14）, central nervous system symptoms 7. 1% （1/14）. Laboratory findings ： decrease of 2 lineages cell in the peripheral blood 100. 0% （ 14/14）, hypefferritinaemia 92. 9% （ 13/14）, hypertrig lyceridemia 21.4% （3/14）, hypofibrinogenemia 35.7% （5/14）, hemophagocytosis in bone marrow 71.4% （ 10/14）, elevated lactate dehydrogenase 64. 3% （9/14）, liver dysfunction 71.4% （10/14）, elevated CRP level 76.8% （11/14）. （3） 12 cases were treated with HLH -2004 protocol, and 2 patients gave up treatment. Conclusion The clinical characteristics of childreng ALL associated haemophagocytic lymphohistiocytosis are diverse, and accompanied by severe ilffection and multiple organ injury, and the patients has extremely poor treatment effect and extremely high mortality.