Acquired Multiple Clotting Factor Inhibitors in Children

Background: Acquired childhood multiple clotting factor inhibitors are rare especially in the absence of lupus anticoagulants. They may represent multiple specific inhibitors or may be non-specific, resulting from molecular mimicry or cross-reacting antibodies. Their exact nature and natural history are not well known. Objective: To report our experience with seven children presenting with prolonged activated partial thromboplastin time (APTT), with or without bleeding, not corrected by mixing, and showing deficiency of > one clotting factor. Design: Prospective review Setting: Mubarak Hospital, Kuwait Subjects: Patients referred to the pediatric hematology unit between 2010 and 2012 with deranged coagulation profiles with or without bleeding, without a previous or family history of a bleeding disorder. They all had multiple clotting factor deficiencies. Intervention: Prothrombin time (PT) and APTT assay Main Outcome Measures: Control of bleeding and normalization of coagulation factors and APTT Results: The patients were aged 6 months to 8 years; three presented with mild to moderate bleeding and five had preceding viral infections. Factor IX was decreased in all cases in addition to deficiencies of factors VIII, X and / or XI in various combinations. There was spontaneous recovery in five patients in whom the factors and APTT normalized within two to five months. One patient died from massive pulmonary hemorrhage and another with nephropathy remains the same after two years. Conclusion: Multiple acquired inhibitors are not uncommon in children, tend to follow viral infections, and are usually transient and not associated with severe bleeding.