Thrombotic microangiopathies in critically ill children: The MATUCIP registry in Spain

Paediatric patients with suspected immune thrombotic thrombocytopenic purpura also experience treatment delays

“…2 In paediatric patients, delayed diagnosis may occur when clinical manifestations overlap with other thrombotic microangiopathies (TMA)-congenital TTP (cTTP) and haemolytic uremic syndrome (HUS). 3,4 When the diagnosis is delayed, it also delays the initiation of therapeutic plasma exchange (TPE). 2,5,6 Where TPE is available, delays may occur due to the need for specialized multiprofessional teams: Expertise is needed for both apheresis and sedation protocols.…”

“…2,6,7 When iTTP occurs in paediatric patients, the presence of a TMA increases suspicion for both HUS and cTTP. 2,4,[7][8][9] Even when a TMA is suspected, other diseases that present with thrombocytopenia are crucial differential diagnosis. 2,4,[7][8][9] In a cohort study of 45 paediatric TTP cases (median age 13 years; range 7-16), common misdiagnoses in 31% of cases (n = 14) included HUS (n = 8), idiopathic thrombocytopenic purpura (n = 3), Evans syndrome (n = 2) or haematological malignancy (n = 1).…”

“…2,4,[7][8][9] Even when a TMA is suspected, other diseases that present with thrombocytopenia are crucial differential diagnosis. 2,4,[7][8][9] In a cohort study of 45 paediatric TTP cases (median age 13 years; range 7-16), common misdiagnoses in 31% of cases (n = 14) included HUS (n = 8), idiopathic thrombocytopenic purpura (n = 3), Evans syndrome (n = 2) or haematological malignancy (n = 1). 2 Even when iTTP is suspected, treatment initiation with TPE is often postponed pending confirmational testing.…”

“…Morgana Pinheiro Maux Lessa 1 Stephen H. Boyle 2 Kate Sanborn 3 Maragatha Kuchibhatla 4 Oluwatoyosi A. Onwuemene 5…”