[Show abstract][Hide abstract] ABSTRACT: Although acquired idiopathic thrombotic thrombocytopenic purpura (ai-TTP) is rare in children, the authors present the case of a 9-month-old boy with ai-TTP showing severe deficiency of ADAMTS13 activity by its inhibitory IgG-autoantibody (4.8 Bethesda units/mL). Plasma exchange therapy was clinically effective but transient. Deficient activity of ADAMTS13 with the presence of its inhibitor persisted for 7 months after the initial diagnosis. However, other laboratory findings improved gradually with steroid (pulse) therapy. The hitherto insufficiently characterized clinical settings of ai-TTP during early childhood underscore the importance of measuring ADAMTS13 activity and its inhibitors for differential diagnosis in patients with thrombocytopenia of unknown etiology.
No preview · Article · Feb 2010 · Pediatric Hematology and Oncology