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补体相关和非补体相关温抗体型自身免疫性溶血性贫血的免疫血液学及临床特征以及预测其发生的危险因素

Immunohematological and Clinical Characterization of Complement and Non-Complement Associated Warm Autoimmune Haemolytic Anemia and Risk Factors Predicting their Occurrences.

作者信息

Das Sudipta Sekhar, Chakrapani Anupam, Bhattacharya Soumya, Biswas Rathindra Nath, Safi Mahammad

机构信息

Department of Transfusion Medicine, Apollo Gleneagles Hospitals, Kolkata, 700054 India.

出版信息

Indian J Hematol Blood Transfus. 2021 Oct;37(4):623-631. doi: 10.1007/s12288-021-01402-3. Epub 2021 Jan 19.

Abstract

Antigen - antibody complexes on heavily coated red cells in Warm autoimmune haemolytic anemia (WAIHA) often activates the complement pathway and red cells bound C3 complement component are encountered in complement associated WAIHA (CWAIHA). Patients belonging to CWAIHA and non-complement associated WAIHA (NCWAIHA) may demographically, clinically and immunohematologically behave differently therefore we planned to study the clinical and immunohematological characteristics of CWAIHA and NCWAIHA with emphasis to various potential factors associated with CWAIHA. The prospective study included 229 patients of WAIHA. Complete DAT evaluation was performed in all these patients. Details of patients and their hematological and biochemical parameters were obtained from patient file and Hospital Information System. In vivo hemolysis was documented as per the criteria established by previous workers. Statistical analysis was done using SPSS statistical package. Of the total 229 patients of WAIHA, 83 (36.2%) belonged to the complement associated WAIHA group. A total of 146 (63.8%) patients were females of which 43 (29.4%) had CWAIHA. The median age of WAIHA patients was 37 years. A total of 46 (56.1%) patients above age 40 years suffered from CWAIHA. Where secondary WAIHA was found in 121 (52.8%) patients; more than half (61.4%) with CWAIHA had underlying aetiology. Over 95% of patients in both categories presented with weakness and pallor. Strong DAT (> 2 +) was observed in 86.7% of CWAIHA patients. Factors like gender, age, aetiology and DAT IgG dilution were independent risk factors for CWAIHA. DAT remained positive even at the end of 10 months of successful treatment. We conclude that detailed characterization of WAIHA with particular emphasis to complement and non-complement associated WAIHA is essential to evaluate the disease characters, immunological behaviours, prognosis and therapeutic management. Moreover an understanding of the risk factors of CWAIHA will help physicians / hematologists and immunohematologists to manage WAIHA more prudently and solicitously.

摘要

温抗体型自身免疫性溶血性贫血(WAIHA)中,重度包被红细胞上的抗原 - 抗体复合物常激活补体途径,在补体相关的WAIHA(CWAIHA)中可发现红细胞结合C3补体成分。CWAIHA患者和非补体相关WAIHA(NCWAIHA)患者在人口统计学、临床和免疫血液学方面可能表现不同,因此我们计划研究CWAIHA和NCWAIHA的临床和免疫血液学特征,并重点关注与CWAIHA相关的各种潜在因素。这项前瞻性研究纳入了229例WAIHA患者。对所有这些患者进行了完整的直接抗人球蛋白试验(DAT)评估。从患者病历和医院信息系统中获取患者及其血液学和生化参数的详细信息。根据先前研究者制定的标准记录体内溶血情况。使用SPSS统计软件包进行统计分析。在229例WAIHA患者中,83例(36.2%)属于补体相关WAIHA组。共有146例(63.8%)患者为女性,其中43例(29.4%)患有CWAIHA。WAIHA患者的中位年龄为37岁。共有46例(56.1%)年龄在40岁以上的患者患有CWAIHA。在121例(52.8%)患者中发现继发性WAIHA;超过一半(61.4%)患有CWAIHA的患者有潜在病因。两类患者中超过95%出现乏力和面色苍白。86.7%的CWAIHA患者观察到强DAT(>2 +)。性别、年龄、病因和DAT IgG稀释度等因素是CWAIHA的独立危险因素。即使在成功治疗10个月后,DAT仍为阳性。我们得出结论,对WAIHA进行详细特征描述,尤其重点关注补体相关和非补体相关WAIHA,对于评估疾病特征、免疫行为、预后和治疗管理至关重要。此外,了解CWAIHA的危险因素将有助于医生/血液学家和免疫血液学家更谨慎、周到地管理WAIHA。

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