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Complement biology for hematologists
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DOI：10.1002/ajh.26855.png)
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Abstract
En 中文
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.
Keywords:
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
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