title: Transient hemorrhagic diathesis associated with an inhibitor of prothrombin with lupus anticoagulant in a 1 1/2-year-old girl: report of a case and review of the literature.
abstract: Acquired inhibitors of coagulation causing bleeding manifestations are rare in children, particularly without an associated underlying disorder such as autoimmune disease. We describe an otherwise healthy 1 1/2-year-old girl who had extensive spontaneous bruising and prolonged bleeding from venipuncture sites. Prothrombin time (PT) and activated partial thromboplastin time (APTT) were prolonged, with evidence of an immediate-acting inhibitor. Thrombin clotting time, fibrinogen, and platelets were normal. Biologic assay of factors II, V, VII, and X were all low, with increasing values at higher dilutions. However, by immunoassay and/or chromogenic assays, only factor II was reduced. An antibody which failed to neutralize prothrombin activity in vitro was detected against radiolabeled prothrombin. Coagulation studies normalized in parallel with clinical recovery and disappearance of the antibody. This case demonstrates acute hypoprothrombinemia-lupus anticoagulant syndrome as a rare presentation of bleeding diathesis in a healthy young child.
authors.affiliation: Department of Pediatrics, New York University Medical Center, New York, New York 10016, USA.
authors.forename: M T
authors.initials: MT
authors.lastname: Lee
authors.forename: M A
authors.initials: MA
authors.lastname: Nardi
authors.forename: J
authors.initials: J
authors.lastname: Hadzi-Nesic
authors.forename: M
authors.initials: M
authors.lastname: Karpatkin
mesh_terms: D000590:Amino Acid Chloromethyl Ketones / Q000494:pharmacology; D000595:Amino Acid Sequence; D000918:Antibody Specificity; D000937:Antigen-Antibody Reactions; D001327:Autoimmune Diseases / Q000209:etiology / Q000276:immunology*; D001780:Blood Coagulation Tests; D003619:Dansyl Compounds / Q000494:pharmacology; D004200:Diseases in Twins; D005170:Factor X / Q000276:immunology; D005260:Female; D006474:Hemorrhagic Disorders / Q000209:etiology / Q000276:immunology*; D006801:Humans; D007223:Infant; D016682:Lupus Coagulation Inhibitor / Q000276:immunology* / Q000302:isolation & purification; D008969:Molecular Sequence Data; D010446:Peptide Fragments / Q000276:immunology; D010612:Pharyngitis / Q000150:complications / Q000276:immunology; D011516:Prothrombin / Q000037:antagonists & inhibitors / Q000276:immunology*; D012075:Remission, Spontaneous; D013917:Thrombin / Q000037:antagonists & inhibitors / Q000276:immunology
chemical_list: D000590:Amino Acid Chloromethyl Ketones; D003619:Dansyl Compounds; D016682:Lupus Coagulation Inhibitor; D010446:Peptide Fragments; C045879:prothrombin fragment 1.2; C030824:dansylglutamyl-glycyl-arginine chloromethyl ketone; D011516:Prothrombin; D005170:Factor X; D013917:Thrombin