伴卡梅现象儿童血管瘤36例临床特点及治疗分析

doi:10.12372/jcp.2024.23e1206

摘要/Abstract

摘要：

目的分析具有卡梅现象的儿童血管瘤患者的临床特点，总结治疗经验。方法回顾性分析2006年4月1日至2021年12月31日就诊的伴有卡梅现象的儿童血管瘤患者的临床资料。结果共36例血管瘤儿童伴有卡梅现象，占同期血管瘤就诊患儿的0.6%。5例（13.9%）患儿在发病早期出现典型卡梅现象，26例（72.2%）肿块位置浅表，瘤体小，4例（11.1%）肿块位于组织深部，瘤体相对较大，1例（2.8%）以便血为首发症状，肿块未被发现。中位随访时间为86个月。所有患儿确诊时中位血小板计数为24.5（11.8~43.5）×10⁹/L，治疗后恢复至168.0（101.8~314.5）×10⁹/L。24例接受综合治疗的患儿均获得完全缓解；12例接受单一治疗的患儿中，5例获得完全缓解，3例部分缓解，4例治疗无效。所有患儿的总体完全缓解率为80.6%，部分缓解率为8.3%，无效率为11.1%；7年总体生存率为88.9%，病死率为11.1%。结论症状隐匿是卡梅现象发病的早期特点。瘤体进行性增大或伴有其他出凝血异常症状的血管瘤患者，需尽早完善相关实验室检查。

关键词: 卡梅现象, 临床特点, 治疗, 儿童

Abstract:

Objective To analyze the clinical characteristics of pediatric hemangioma patients with Kasabach-Merritt phenomenon (KMP), and sum up its treatment experience. Methods The clinical data of children with KMP diagnosed from April 1, 2006 to December 31, 2021 were retrospectively analyzed. Results A total of 36 children with hemangioma were accompanied by KMP, accounting for 0.6% of the children with hemangioma. Five patients (13.9%) showed typical clinical manifestations of KMP in the early stage of the disease; 26 patients (72.2%) had superficial tumors with small lesions; 4 patients (11.1%) had deep-seated tumors with relatively larger lesions; 1 patient (2.8%) presented with hematochezia as the initial symptom, and the tumor was not detected. Median follow-up time was 86 months. All patients manifested thrombocytopenia at the time of diagnosis and the median platelet counts were 24.5(11.8-43.5)×10⁹/L, which increased to 168.0(101.8-314.5)×10⁹/L after treatment. Twenty-four patients (66.7%) received combined therapy, all of them got complete remission (CR). Twelve patients (33.3%) received monotherapy, of them, five patients got CR, three got partial remission (PR), four showed no response. The overall CR rate was 80.6%, PR rate was 8.3%, and NR rate was 11.1%. The 7-year overall survival rate was 88.9% and the fatality rate was 11.1%. Conclusions The masking of symptom was the characteristics of KMP. Appropriate laboratory testing should be performed as soon as possible for patients with dysfunction of blood coagulation or rapidly progressive hemangiomas.

Key words: Kasabach-Merritt phenomenon, clinical characteristics, treatment, child

黄世浩, 袁晓军. 伴卡梅现象儿童血管瘤36例临床特点及治疗分析[J]. 临床儿科杂志, 2024, 42(11): 917-921.

HUANG Shihao, YUAN Xiaojun. Analysis on clinical characteristics and treatment for Kasabach-Merritt phenomenon in 36 children with hemangioma[J]. Journal of Clinical Pediatrics, 2024, 42(11): 917-921.

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参考文献 14

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检验项目	治疗前(n=36)	治疗后(n=36)	统计量	P
白细胞计数[M(P₂₅~P₇₅)]/×10⁹·L^-1	7.6(6.1~9.9)	9.8(7.6~12.3)	Z=2.10	0.036
血红蛋白($\bar{x} \pm s$)/g·L^-1	96.0±19.0	112.0±15.0	t=4.16	<0.001
血小板计数[M(P₂₅~P₇₅)]/×10⁹·L^-1	24.5(11.8~43.5)	168.0(101.8~314.5)	Z=6.08	<0.001
凝血酶原时间[M(P₂₅~P₇₅)]/s	11.5(10.9~12.8)	12.0(10.9~12.8)	Z=0.23	0.822
部分活性凝血酶原时间[M(P₂₅~P₇₅)]/s	37.5(31.2~42.4)	35.5(30.8~37.2)	Z=1.48	0.138
凝血酶时间[M(P₂₅~P₇₅)]/s	19.3(17.2~23.2）	16.4(15.4~17.1)	Z=3.93	<0.001
纤维蛋白原[M(P₂₅~P₇₅)]/g·L^-1	1.0(0.8~1.4)	2.3(2.0~2.5)	Z=4.07	<0.001
D-Ⅱ聚体[M(P₂₅~P₇₅)]/mg·L^-1	6.2(4.4~12.7)	0.3(0.2~0.5)	Z=5.39	<0.001

治疗方式	具体方案	例数	疗效	预后
单纯手术	-	1	CR	长期存活
单纯药物	甲基泼尼松龙	2	CR	长期存活
	甲基泼尼松龙	2	PR	长期存活
	普萘洛尔	1	NR	死亡
	干扰素	1	NR	死亡
	甲基泼尼松龙+长春新碱	2	CR	长期存活
	甲基泼尼松龙+长春新碱	2	NR	死亡
	甲基泼尼松龙+普萘洛尔	1	CR	长期存活
综合治疗	药物+介入栓塞+维持治疗	24	CR	长期存活

项目	综合治疗组(n=24)	单纯药物治疗组(n=11)	统计量	P
凝血酶原时间($\bar{x} \pm s$)/s	11.8±1.0	20.5±15.8	t=2.67	0.012
凝血酶时间($\bar{x} \pm s$)/s	16.0±1.0	27.5±18.6	t=3.02	0.005
部分活性凝血酶原时间($\bar{x} \pm s$)/s	34.0±3.5	44.1±17.5	t=2.72	0.011
D-Ⅱ聚体[M(P₂₅~P₇₅)]/mg·L^-1	0.3(0.2~0.4)	0.4(0.3~10.6)	Z=1.26	0.221
纤维蛋白原($\bar{x} \pm s$)/g·L^-1	2.3±0.4	1.6±1.1	t=3.15	0.004
血小板计数[M(P₂₅~P₇₅)]/×10⁹·L^-1	228.0(144.5~356.8)	91.0(24.0~106.5)	Z=3.66	<0.001
缓解[n(%)]	24(100.0)	7(63.6)	-	0.002¹⁾

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