McLeod Bruce C
Blood Center, Rush University Medical Center, Chicago, Illinois 60612, USA.
Curr Opin Hematol. 2007 Nov;14(6):647-54. doi: 10.1097/MOH.0b013e3282c8ca66.
This article reviews recent publications that bear on the evidential basis for therapeutic apheresis in diseases in which hemolytic anemia is a prominent feature.
Therapeutic plasma exchange continues to be reported sporadically in severe autoimmune hemolytic anemia, with inconsistent results. Autoimmune deficiency of ADAMTS13 has provided a compelling rationale for therapeutic plasma exchange in some patients with thrombotic thrombocytopenic purpura; conversely such deficiency is consistently absent in certain clinically similar syndromes for which therapeutic plasma exchange is not or may not be beneficial. Refinements in assays for ADAMTS13 should further clarify its role in idiopathic thrombotic thrombocytopenic purpura. Oral iron chelators have shown promise in recent trials in chronically transfused patients with sickle cell disease and may provide an alternative to red cell exchange to prevent iron overload.
The proper role of therapeutic plasma exchange in the treatment of autoimmune hemolytic anemia remains uncertain. Therapeutic plasma exchange continues to be indicated for idiopathic thrombotic thrombocytopenic purpura regardless of ADAMTS13 levels, but more accessible and physiological ADAMTS13 assays may raise questions about the rationale for and value of plasma exchange in ADAMTS13 nondeficient patients. Oral iron chelation may obviate the need for red cell exchange as a means to prevent iron overload in chronically transfused patients with sickle cell disease.
本文回顾了近期有关以溶血性贫血为显著特征的疾病中治疗性血液成分单采的循证医学依据的出版物。
治疗性血浆置换在严重自身免疫性溶血性贫血中的报道仍较为零散，结果不一。ADAMTS13自身免疫缺陷为某些血栓性血小板减少性紫癜患者进行治疗性血浆置换提供了令人信服的理论依据；相反，在某些临床相似的综合征中，尽管进行了治疗性血浆置换但并无益处，而这些综合征中始终不存在这种缺陷。ADAMTS13检测方法的改进应能进一步阐明其在特发性血栓性血小板减少性紫癜中的作用。口服铁螯合剂在近期针对镰状细胞病慢性输血患者的试验中显示出前景，可能为预防铁过载提供红细胞置换的替代方法。
治疗性血浆置换在自身免疫性溶血性贫血治疗中的恰当作用仍不确定。无论ADAMTS13水平如何，治疗性血浆置换仍适用于特发性血栓性血小板减少性紫癜，但更易获得且更具生理性的ADAMTS13检测方法可能会引发关于ADAMTS13不缺乏患者进行血浆置换的理论依据和价值的问题。口服铁螯合可能无需进行红细胞置换来预防镰状细胞病慢性输血患者的铁过载。
