Neonatal exchange transfusion: hereditary thrombotic thrombocytopenic purpura (hTTP) should be in the differential diagnosis

Introduction Transition from intra- to extra-uterine life is complex and abnormalities in this adaptive process may require resuscitation and/or intensive treatment in the first days of life, which seems to be particularly true for neonates later in life diagnosed with hTTP. These neonates may show severe hyperbilirubinemia, profound anemia and thrombocytopenia, requiring emergency treatment including neonatal exchange transfusion (NExT) in the more complicated cases. Severe congenital ADAMTS13 deficiency is the cause of hTTP, a rare autosomal recessively inherited disorder rarely diagnosed at birth despite of indicative symptoms and laboratory parameters.