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成年原发性自身免疫性溶血性贫血患者溶血的临床特征、严重程度及其对类固醇的反应:来自单一机构的前瞻性队列研究

Clinical Profile and Severity of Hemolysis in Adult Patients of Primary Autoimmune Hemolytic Anemia and Their Response to Steroid: A Prospective Cohort Study from Single Institution.

作者信息

Ray Gopal Krushna, Mishra Debasish, Jena Rabindra Kumar, Mahapatra Smita, Palai Sabita, Parida Ansuman Abhishek

机构信息

Department of Transfusion Medicine, Sriram Chandra Bhanja Medical College and Hospital, Cuttack, Odisha India.

Department of Clinical Hematology, Sriram Chandra Bhanja Medical College and Hospital, Cuttack, Odisha India.

出版信息

Indian J Hematol Blood Transfus. 2021 Jan;37(1):119-125. doi: 10.1007/s12288-020-01326-4. Epub 2020 Aug 7.

Abstract

Autoimmune hemolytic anaemia (AIHA) has traditionally been classified based on the temperature sensitivity of the autoagglutinins as warm (WAIHA), cold (CAIHA) and mixed type. Autoagglutinin may be of IgG or IgM type. The present prospective study was conducted to evaluate the profile of clinical picture, severity of haemolysis, treatment response of steroid. This study on patients of adult primary AIHA was conducted by taking complete history followed by detail physical examination. Laboratory investigations were performed to establish haemolytic anaemia and to assess severity of haemolysis. Immunehematological work up including blood grouping, direct antiglobulin test (DAT), IAT, antibody screening, adsorption elution was performed to diagnose type of AIHA. All cases were followed up to assess the response to prednisolone. All the data were collected and analysed by SPSS 19. Out of 62 primary AIHA cases, female were affected more than male (41:21). WAIHA is most common type (42, 67.8%) followed by mixed (20.9%) and cold AIHA (11.3%). Severity of haemolysis showed significant correlation with the DAT strength and not with type of AIHA. ( < 0.05) On oral prednisolone, 22 cases attended complete remission, while relapse, drug dependency and partial remission was achieved in 13, 9, 3 cases respectively. Severity of haemolysis in AIHA is directly related with DAT strength. WAIHA is most common type and can be managed with oral prednisolone (cr 45.2%), without red cell transfusion in most of cases. Mixed type AIHA cases were presented mostly with severe haemolysis, with minimum therapeutic response to prednisolone and maximum relapse/drug dependency.

摘要

自身免疫性溶血性贫血(AIHA)传统上是根据自身凝集素的温度敏感性分为温抗体型(WAIHA)、冷抗体型(CAIHA)和混合型。自身凝集素可能是IgG型或IgM型。本前瞻性研究旨在评估临床表现、溶血严重程度、类固醇治疗反应。本研究针对成年原发性AIHA患者,先进行完整病史采集,然后进行详细体格检查。进行实验室检查以确诊溶血性贫血并评估溶血严重程度。进行包括血型鉴定、直接抗球蛋白试验(DAT)、间接抗球蛋白试验(IAT)、抗体筛查、吸附洗脱在内的免疫血液学检查以诊断AIHA类型。所有病例均进行随访以评估对泼尼松龙的反应。所有数据采用SPSS 19进行收集和分析。在62例原发性AIHA病例中,女性受累多于男性(41:21)。WAIHA是最常见类型(42例,67.8%),其次是混合型(20.9%)和冷抗体型AIHA(11.3%)。溶血严重程度与DAT强度显著相关,而与AIHA类型无关(P<0.05)。口服泼尼松龙后,22例完全缓解,13例、9例、3例分别出现复发、药物依赖和部分缓解。AIHA的溶血严重程度与DAT强度直接相关。WAIHA是最常见类型,多数病例口服泼尼松龙(完全缓解率45.2%)即可治疗,无需红细胞输注。混合型AIHA病例大多表现为严重溶血,对泼尼松龙治疗反应最小,复发/药物依赖最多。

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本文引用的文献

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Hemolytic anemia due to warm autoantibodies.温抗体型自身免疫性溶血性贫血
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Approaches to selecting blood for transfusion to patients with autoimmune hemolytic anemia.
Transfusion. 2002 Nov;42(11):1390-2. doi: 10.1046/j.1537-2995.2002.00284.x.

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