Chaudhary R K, Das Sudipta Sekhar
Department of Transfusion Medicine, SGPGIMS, Lucknow, Uttar Pradesh, India.
Department of Transfusion Medicine, Apollo Gleneagles Hospitals, Kolkata, West Bengal, India.
Asian J Transfus Sci. 2014 Jan;8(1):5-12. doi: 10.4103/0973-6247.126681.
Autoimmune hemolytic anemia (AIHA) is not an uncommon clinical disorder and requires advanced, efficient immunohematological and transfusion support. Many AIHA patients have underlying disorder and therefore, it is incumbent upon the clinician to investigate these patients in detail, as the underlying condition can be of a serious nature such as lymphoproliferative disorder or connective tissue disorder. Despite advances in transfusion medicine, simple immunohematological test such as direct antiglobulin test (DAT) still remains the diagnostic hallmark of AIHA. The sensitive gel technology has enabled the immunohematologist not only to diagnose serologically such patients, but also to characterize red cell bound autoantibodies with regard to their class, subclass and titer in a rapid and simplified way. Detailed characterization of autoantibodies is important, as there is a relationship between in vivo hemolysis and strength of DAT; red cell bound multiple immunoglobulins, immunoglobulin G subclass and titer. Transfusing AIHA patient is a challenge to the immunohematologist as it is encountered with difficulties in ABO grouping and cross matching requiring specialized serological tests such as alloadsorption or autoadsorption. At times, it may be almost impossible to find a fully matched unit to transfuse these patients. However, transfusion should not be withheld in a critically ill patient even in the absence of compatible blood. The "best match" or "least incompatible units" can be transfused to such patients under close supervision without any serious side-effects. All blood banks should have the facilities to perform the necessary investigations required to issue "best match" packed red blood cells in AIHA. Specialized techniques such as elution and adsorption, which at times are helpful in enhancing blood safety in AIHA should be established in all transfusion services.
自身免疫性溶血性贫血(AIHA)是一种并不罕见的临床病症,需要先进、高效的免疫血液学和输血支持。许多AIHA患者存在潜在疾病,因此,临床医生有责任对这些患者进行详细检查,因为潜在病症可能性质严重,如淋巴增殖性疾病或结缔组织疾病。尽管输血医学取得了进展,但诸如直接抗球蛋白试验(DAT)等简单的免疫血液学检测仍然是AIHA的诊断标志。灵敏的凝胶技术使免疫血液学家不仅能够从血清学上诊断此类患者,还能快速、简便地对红细胞结合的自身抗体进行分类、亚类和滴度鉴定。自身抗体的详细鉴定很重要,因为体内溶血与DAT强度、红细胞结合的多种免疫球蛋白、免疫球蛋白G亚类和滴度之间存在关联。给AIHA患者输血对免疫血液学家来说是一项挑战,因为在ABO血型分组和交叉配血时会遇到困难,需要进行诸如同种吸附或自身吸附等专门的血清学检测。有时,几乎不可能找到完全匹配的单位给这些患者输血。然而,即使没有相容的血液,对于重症患者也不应停止输血。在密切监测下,可以给此类患者输注“最佳匹配”或“最不相容单位”的血液,而不会产生任何严重的副作用。所有血库都应具备进行必要检测的设施,以便在AIHA患者中发放“最佳匹配”的浓缩红细胞。所有输血服务机构都应建立诸如洗脱和吸附等专门技术,这些技术有时有助于提高AIHA患者的输血安全性。