61例血栓性血小板减少性紫癜患者的临床分析

doi:10.19723/j.issn.1671-167X.2021.01.032

摘要/Abstract

摘要：

目的: 对61例血栓性血小板减少性紫癜(thrombotic thrombocytopenic purpura, TTP)的临床特征、诊断、治疗及转归进行分析总结,提高临床诊治水平。方法: 回顾性分析北京大学人民医院2004年1月至2019年3月收治的61例TTP患者的临床资料,观察这些患者的临床表现、血常规、溶血指标及血管性血友病因子裂解酶(von Willebrand factor-cleaving protease, vWF-cp,又称ADAMTS13)。根据离院时的结局分为存活组与死亡组,比较两组患者的临床特点、中性粒细胞-淋巴细胞比值(neutrophil to lymphocyte ratio, NLR)和血浆置换的差异。计算PLASMIC评分,与ADAMTS13分组比较,判断PLASMIC评分预测ADAMTS13的准确性。结果: 61例TTP患者中,男性22例,女性39例,平均年龄(48±17)岁。五联征表现者48例,仅有三联征者9例,其余4例无神经精神症状。死亡27例(44.3%),存活34例(55.7%)。61例TTP患者的血小板(12.9±9.5)×10⁹/L,血红蛋白(66.5±20.7) g/L,外周血破碎红细胞3%(2%,7%),血浆游离血红蛋白升高为360(200,457) mg/L,乳酸脱氢酶1 508(811,2134) U/L,出凝血方面基本正常。30例患者的ADAMTS13值49.0(40.8,61.3) μg/L,10例患者ADAMTS活性<5%,其余21例未查。PLASMIC评分为6~7分者58例,5分者2例,4分者1例。PLASMIC预测ADAMTS13减少或活性降低的灵敏度高达97.5%。死亡组NLR较存活组高,但二者差异无统计学意义(P>0.05)。死亡组血浆置换(plasma exchange,PEX)总量和次数均较存活组显著减少,二者差异有统计学意义(P<0.05)。两组患者在糖皮质激素、人免疫球蛋白治疗方面差异无统计学意义(P>0.05)。结论: PEX能明显提高TTP患者存活率。PLASMIC评分可方便、快捷地预测ADAMTS13活性降低的可能性,利于TTP的及早诊断和PEX治疗。NLR可反映全身炎症过程,但在TTP中的意义有待进一步研究。

关键词: 血栓性血小板减少性紫癜, 血管性血友病因子裂解酶, 血浆置换

Abstract:

Objective: To analyze and summarize the clinical features, diagnosis, treatment and prognosis of 61 patients with thrombotic thrombocytopenic purpura (TTP),so as to improve the ability of diagnosis and treatment.Methods: The clinical data of 61 TTP patients admitted to Peking University People’s Hospital from January 2004 to March 2019 were retrospectively analyzed, and the clinical manifestations, blood routine, hemolysis indicators, and von Willebrand factor lyase (von Willebrand factor-cleaving protease, vWF-CP, also known as ADAMTS13) of these patients were observed. According to the outcome at the time of discharge, they were divided into survival group and death group, and the differences in clinical characteristics, neutrophil to lymphocyte ratio (NLR) and plasma exchange between the two groups were compared. The PLASMIC scores were calculated and compared with ADAMTS13 to determine the accuracy of the PLASMIC score in predicting ADAMTS13.Results: Among the 61 TTP patients, 22 were males and 39 were females, with an average age of (48±17) years. In the study, 48 cases had pentalogy, only 9 had triad, and the remaining 4 had no neuropsychiatric symptoms. Twenty-seven cases (44.3%) died and 34 cases (55.7%) survived. Among the 61 TTP patients, the platelet count was (12.9±9.5)×10⁹/L, the hemoglobin (66.5±20.7) g/L, the percentage of erythrocyte fragments 3% (2%, 7%), and the plasma free hemoglobin increased to 360 (200, 457) mg /L, and the lactate dehydrogenase 1 508 (811, 2 133.8) U/L. The blood clotting was basically normal. The ADAMTS13 value of 30 patients was 49.0 (40.8, 61.3) μg/L, the ADAMTS activity of 10 patients was<5%, and the remaining 21 patients were not checked. The PLASMIC score was 6-7 in 58 cases, 5 in 2 cases, and 4 in 1 case. The PLASMIC score predicted the decreased activity or the reduction of ADAMTS with a sensitivity as high as 97.5%. The NLR in the death group was higher than that in the survival group, but the difference was not statistically significant (P>0.05). The total amount and frequency of plasma exchange (PEX) in the death group were significantly less than those in the survival group, and the difference was statistically significant (P<0.05). There was no significant difference in the treatment of glucocorticoids and human immunoglobulin between the two groups (P>0.05). Conclusion: PEX can significantly improve the survival rate of TTP patients. PLASMIC score can easily and quickly predict the possibility of ADAMTS13 activity reduction, which is beneficial to the early diagnosis of TTP and PEX treatment. NLR can reflect the systemic inflammatory process, but its significance in TTP needs further study.

Key words: Thrombotic thrombocytopenic purpura, Von willebrand factor-cleaving protease, Plasma exchange

中图分类号:

R558.2

石茂静,高伟波,黄文凤,朱继红. 61例血栓性血小板减少性紫癜患者的临床分析[J]. 北京大学学报（医学版）, 2021, 53(1): 210-214.

SHI Mao-jing,GAO Wei-bo,HUANG Wen-feng,ZHU Ji-hong. Clinical analysis of 61 patients with thrombotic thrombocytopenic purpura[J]. Journal of Peking University (Health Sciences), 2021, 53(1): 210-214.

图/表 4

表1

表2

61例TTP患者实验室检查结果"

Items	Value
Platelet/(10⁹/L), $x - ± s$		12.9±9.59
Hemoglobin /(g/L), $x - ± s$		66.5±20.7
LDH /(U/L), M(P₂₅, P₇₅)		1 508 (811, 2 134)
Creatinine/(μmol/L), M(P₂₅, P₇₅)		105.0 (74.0, 157.5)
Plasma free hemoglobin/(mg/L), M(P₂₅, P₇₅)		360.0 (200.0, 457.0)
Prothrombin time/s, $x - ± s$		13.3±7.0
Activated partial thrombopladtin time/s, $x - ± s$		29.8±5.1
D-Dimer/(mg/L), M(P₂₅, P₇₅)		0.89 (0.40, 1.35)
Fibrinogen/(mg/L), $x - ± s$		2 812±1 001
NLR, M(P₂₅, P₇₅)		3.4 (2.1, 5.7)

表2

表3

表4

参考文献 17

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Items	n	Incidence rate/%
Thrombocytopenia	61	100.0
MAHA	61	100.0
Neuropsychiatric symptoms	57	93.4
Fever	58	95.1
Renal impairtment	54	88.5

Items	Death group	Survival group	Z/t	P value
Age/years	54.9±16.7	42.4±14.8	3.219	0.002
PEX/times	0 (0, 3)	4 (2, 6)	-3.436	0.001
PEX volume dose/mL	0 (0, 4 000)	6 500 (2 650, 11 350)	-3.327	0.001
Platelet/(10⁹/L)	10.5 (6.2, 16.3)	8.5 (6.2, 13.5)	-0.456	0.648
Hemoglobin/(g/L)	62.8±19.9	69.5±21.4	-1.188	0.240
LDH/(U/L)	1 519 (838, 2 205)	1 423 (774, 1 828)	-0.495	0.621
Creatinine/(μmol/L)	112.0 (83.0, 210.0)	82.5 (65.8, 130.0)	-2.309	0.021
NLR	4.0 (2.8, 5.9)	3.6 (2.0, 5.5)	-0.418	0.676