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基线荧光抗核抗体阳性对原发性自身免疫性溶血性贫血患者临床结局的影响。

Impact of baseline fluorescent antinuclear antibody positivity on the clinical outcome of patients with primary autoimmune hemolytic anemia.

作者信息

Rangnekar Aseem, Shenoy M Suchitra, Mahabala Chakrapani, Balanthimogru Prashantha

机构信息

Assistant professor, Department of Microbiology, Gandhi Medical College, Bhopal, Madhya Pradesh, India.

Professor and Head, Department of Microbiology, Kasturba Medical College, Mangalore, Manipal Academy of Higher Education, Manipal, India.

出版信息

Hematol Transfus Cell Ther. 2023 Apr-Jun;45(2):204-210. doi: 10.1016/j.htct.2022.01.013. Epub 2022 Mar 4.

DOI:10.1016/j.htct.2022.01.013
PMID:35307304
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10244249/
Abstract

INTRODUCTION

Autoimmune haemolytic anaemia (AIHA) is an autoimmune disorder that can present in primary or secondary forms. The literature looking at impact of baseline fluorescent antinuclear antibody (FANA) positivity on outcomes of AIHA patients is infrequent.

OBJECTIVE

To study the impact of baseline FANA positivity in patients with primary AIHA.

METHOD

A prospective cohort study involving 29 consecutive primary AIHA patients presenting to the Haematology department from 2013 to 2015 was analysed. After recording baseline investigations including fluorescent ANA, all patients were treated as per the standard therapeutic protocols. Clinical remission, disease free survival, relapse, mortality were compared between the FANA positive and FANA Negative AIHA groups.

RESULTS

Baseline FANA positivity was found in 17 patients (58.62%). Both the groups were comparable in terms of age, sex, Hemoglobin, LDH at presentation, number of lines of treatment needed and duration of follow up. Evan's syndrome was seen in six of FANA positive patients which was statistically significant (0 v/s 6, p = 0.023). FANA positive patients had significantly higher rates of relapse per patient month follow up (1.22 v/s 3.57, p = 0.023) and lower rates of complete response (83.33% v/s 35.29%, p = 0.0118) and relapse free survival at five years. Morbidity and mortality were numerically higher in FANA positive patients.

CONCLUSION

Baseline FANA positivity among AIHA patients was found to be associated with lower complete response rates and higher relapse rates with possible higher rates of morbidity. Presence of FANA will give us prognostic value and help us in deciding the treatment options.

摘要

引言

自身免疫性溶血性贫血(AIHA)是一种自身免疫性疾病,可呈原发性或继发性形式。关于基线荧光抗核抗体(FANA)阳性对AIHA患者预后影响的文献较少。

目的

研究基线FANA阳性对原发性AIHA患者的影响。

方法

对2013年至2015年连续就诊于血液科的29例原发性AIHA患者进行前瞻性队列研究。记录包括荧光ANA在内的基线检查后,所有患者均按照标准治疗方案进行治疗。比较FANA阳性和FANA阴性AIHA组之间的临床缓解、无病生存、复发、死亡率。

结果

17例患者(58.62%)基线FANA阳性。两组在年龄、性别、就诊时血红蛋白、乳酸脱氢酶、所需治疗线数和随访时间方面具有可比性。FANA阳性患者中有6例出现伊文氏综合征,具有统计学意义(0例对6例,p = 0.023)。FANA阳性患者每患者月随访的复发率显著更高(1.22对3.57,p = 0.023),完全缓解率更低(83.33%对35.29%,p = 0.0118),且五年无复发生存率更低。FANA阳性患者的发病率和死亡率在数值上更高。

结论

发现AIHA患者中基线FANA阳性与较低的完全缓解率和较高的复发率相关,可能发病率也更高。FANA的存在将为我们提供预后价值,并帮助我们决定治疗方案。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/664f/10244249/117a929374ef/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/664f/10244249/117a929374ef/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/664f/10244249/117a929374ef/gr1.jpg

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