Coagulation Failure in Babies with Rhesus Isoimmunization

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Abstract
Serial prospective studies of coagulation status have been undertaken on 73 babies with a positive Coombs test. No abnormalities were detected in the babies with mild hemolytic disease, but 7 of the 36 babies with severe hemolytic disease (cord Hb 85 .mu.mol/l) showed evidence of transient defibrination I day after birth and another 6 had evidence of coagulation failure at birth with a platelet count of less than 150 .times. 109/l and a severe deficiency of multiple coagulation factors. The level of factor II and factor X was less than 1/5 of the normal cord blood level in these 6 babies and the level of I, VII and IX was severely reduced. The factor VIII level was normal or high. Exchange transfusion started within 1 h of birth corrected the immediate factor deficiency in these 6 babies, but evidence of defibrination then became apparent with afibrinogenemia, a marked fall in factors II and V, less constant falls in factors VII, IX and X, and a raised fibrin:fibrinogen degradation product level. One of these 6 babies died with severe pulmonary hypoplasia within an hour of birth; the other 5 died from hemorrhage into the lung or brain 1 1/2-6 days after birth. The very low vitamin-K dependent factor levels in the cord blood of the babies who died are presumably the result of liver damage in utero, but the subsquent changes are those of a consumption coagulopathy. Simple screening tests at birth served to indicate which babies were at risk. Death due to hemorrhage might be reduced by more intensive factor replacement before there is overt evidence of hemorrhage in these babies.