关键词：
Animals
Factor VIII/analysis
Hemophilia A/blood
Humans
Immune Sera
Macromolecular Substances
Molecular Weight
Platelet Aggregation
Rabbits/immunology
von Willebrand Diseases/blood
摘要：
Antihemophilic factor (AHF, factor VIII) is a macromolecule that can be dissociated into 2 subcomponents. One, of high molecular weight (MW), forms precipitates with heterologous antiserum against AHF and supports ristocetin-induced aggregation of platelets. The other, of lower MW, has procoagulant activity. The plasma of patients with classic hemophilia contains a form of AHF with properties resembling those of the high MW subcomponent. Studies of Hoyer and Breckenridge have suggested that the low MW subcomponent is present in a minority of patients whose plasmas inhibit human circulating anticoagulant antibodies against AHF. Such individuals, CRM+ [cross reacting material], have a species of AHF possessing a nonfunctional variant of the low MW subcomponent. CRM+ hemophilic plasma or serum, like the low MW subcomponent of normal AHF, blocked the inhibitory properties of circulating anticoagulants, in contrast to the high MW subcomponent of normal AHF. A nonfunctional low MW subcomponent was identified in partially purified AHF-like material separated from the plasmas of both CRM+ and CRM- hemophiliacs, the latter being patients whose plasmas do not neutralize circulating anticoagulants. This low MW subcomponent, like that of normal AHF, inhibited antiserum against the normal low MW subcomponent, but not antiserum against the normal high MW subcomponent. The defect in the hemophilic plasmas tested was probably not associated with the absence of the low MW subcomponent of AHF.
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MEDLINE期刊
