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IgA 相关性自身免疫性溶血性贫血的临床观点、免疫血液学见解和输血管理。

Clinical perspectives, immunohematologic insights, and transfusion management in IgA-associated autoimmune hemolytic anemia.

机构信息

Transfusion Medicine, Apollo Multispeciality Hospitals Ltd., 58 Canal Circular Road, Kadapara, Phool Bagan, Kankurgachi, Kolkata, West Bengal, India, 700054.

Transfusion Medicine, Apollo Multispeciality Hospitals Ltd., Kolkata, India.

出版信息

Immunohematology. 2022 Sep 22;38(3):82-89. doi: 10.21307/immunohematology-2022-047. Print 2022 Sep 1.

Abstract

Autoimmune hemolytic anemia (AIHA) due to warm-reacting IgA autoantibodies is rare. Here, we explored the clinical and immunohematologic characteristics of patients suffering from IgA-associated warm AIHA (WAIHA) and their transfusion management. The 9-year study included 214 patients with WAIHA who were further classified into two groups: (1) IgA-associated WAIHA and (2) non-IgA-associated WAIHA. Clinical and laboratory details were obtained from patient files and the Hospital Information System. All immunohematologic investigations were performed following standard operating procedures and established protocols. Among the 214 patients with WAIHA, 17 (7.9%) belonged to the IgA-associated group; of these, two IgA-only WAIHA cases were found. The mean hemoglobin in this group was 5.58 g/dL, and 15 (88.2%) of these patients received a total of 32 units of packed red blood cell (RBC) transfusions. hemolytic markers were significantly abnormal in the IgA-associated WAIHA group when compared with the non-IgA group. Secondary WAIHA was found in 11 (64.7%) patients with IgA-associated WAIHA. Patients with IgA-associated WAIHA received more blood transfusions than individuals in the non-IgA group ( = 0.0004). A total of 17 (7.9%) patients with WAIHA experienced adverse events to blood transfusion. Detailed characterization of WAIHA with particular emphasis on IgA-associated and non-IgA-associated WAIHA is essential to evaluate the disease characteristics, access the degree of hemolysis, understand the immunohematologic behaviors of the antibodies, and manage blood transfusions.

摘要

自身免疫性溶血性贫血(AIHA)由热反应性 IgA 自身抗体引起的情况较为少见。在此,我们探讨了患有 IgA 相关性温抗体型自身免疫性溶血性贫血(WAIHA)患者的临床和免疫血液学特征及其输血管理。这项为期 9 年的研究纳入了 214 例 WAIHA 患者,进一步分为两组:(1)IgA 相关性 WAIHA;(2)非 IgA 相关性 WAIHA。临床和实验室详细信息从患者档案和医院信息系统中获取。所有免疫血液学检查均按照标准操作规程和既定方案进行。在 214 例 WAIHA 患者中,有 17 例(7.9%)属于 IgA 相关性组;其中,发现了 2 例仅 IgA 相关性 WAIHA 病例。该组的平均血红蛋白水平为 5.58 g/dL,其中 15 例(88.2%)患者共接受了 32 个单位的浓缩红细胞(RBC)输血。与非 IgA 组相比,IgA 相关性 WAIHA 组的溶血标志物明显异常。在 11 例(64.7%)IgA 相关性 WAIHA 患者中发现了继发性 WAIHA。与非 IgA 组相比,IgA 相关性 WAIHA 患者接受了更多的输血( = 0.0004)。共有 17 例(7.9%)WAIHA 患者发生了输血不良反应。详细描述 WAIHA,特别强调 IgA 相关性和非 IgA 相关性 WAIHA,对于评估疾病特征、评估溶血程度、了解抗体的免疫血液学行为以及管理输血非常重要。

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