Acquired symptomatic inhibitors of plasma clotting factors in nonhemophilic children.
Three children presenting with severe symptomatic bleeding episodes (one child developing a life-threatening subdural hematoma) due to acquired coagulation inhibitors are reported. In two patients, there was a history of an antecedent viral syndrome. None had evidence of drug exposure or an underlying immune disorder. All responded to the administration of corticosteroids, although one patient was steroid-dependent and required immunosuppressive therapy to achieve a complete response. Laboratory characteristics did not clearly distinguish the type of circulating inhibitor present in these children. Since bleeding was a prominent feature, we suggest that the inhibitors noted represent multiple specific coagulation factor inhibitors, rather than a "lupus-like" inhibitor which represents a common antibody to phospholipid-dependent coagulation tests. Such inhibitors may not be as benign as the common lupus anticoagulant seen in adult patients and should be considered in the child with symptomatic bleeding and a prolonged partial thromboplastin time. The true incidence and exact mechanism of action of acquired inhibitors in children has not been established.
D001777:Blood Coagulation; D001779:Blood Coagulation Factors / Q000032:analysis*; D002675:Child, Preschool; D005260:Female; D006406:Hematoma / Q000188:drug therapy / Q000209:etiology*; D006801:Humans; D008198:Lymph Nodes / Q000473:pathology; D008206:Lymphatic Diseases / Q000175:diagnosis* / Q000209:etiology / Q000276:immunology; D008214:Lymphocytes / Q000276:immunology; D008297:Male; D010314:Partial Thromboplastin Time; D011241:Prednisone / Q000627:therapeutic use
D001779:Blood Coagulation Factors; D011241:Prednisone