Complement biology for hematologists

被引：16

作者：

Duval, Anna ^{[1
,2
]}

Fremeaux-Bacchi, Veronique ^{[1
,3
]}

机构：

[1] Ctr Rech Cordeliers, Inserm, UMR S1138, Paris, France

[2] Univ Hosp Strasbourg, Dept Nephrol Dialysis & Transplantat, Strasbourg, France

[3] Hop Europeen Georges Pompidou, Assistance Publ Hop Paris, Serv Immunol, Paris, France

来源：

AMERICAN JOURNAL OF HEMATOLOGY | 2023年 / 98卷

关键词：

PAROXYSMAL-NOCTURNAL HEMOGLOBINURIA; THROMBOTIC THROMBOCYTOPENIC PURPURA; HEMOLYTIC-UREMIC SYNDROME; SICKLE-CELL-DISEASE; CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME; FACTOR-H; IMMUNE THROMBOCYTOPENIA; INHIBITOR ECULIZUMAB; TRANSFUSION REACTION; TERMINAL COMPLEMENT;

D O I：

10.1002/ajh.26855

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

The complement system is part of the innate immunity. An increased activation or a loss of the regulation of this fine-tuned cascade is involved in a variety of hematological diseases. During the last decade, anti-C5 therapies have revolutionized the management and prognosis of paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic and uremic syndrome (aHUS). The availability of a rapidly growing number of innovative complement inhibitors has opened new therapeutic perspectives for several other hematological disorders in which the complement is involved at different degrees. This review focuses on complement biology and its mechanisms of activation in hematological diseases.

引用

页码：S5 / S19

页数：15

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