A diagnostic dilemma occurred when thrombotic microangiopathy developed during pregnancy. The diagnostic criteria of thrombotic microangiopathy include thrombocytopenia (platelets <100) and microangiopathic haemolytic anaemia (including thrombotic thrombocytopenic purpura and haemolytic-uraemic syndrome). An urgent interdisciplinary approach is required to treat thrombotic microangiopathy in pregnancy to differentiate between thrombotic microangiopathy and HELLP syndrome (haemolysis, elevated liver enzymes, low platelets). This case presented with the pentad of thrombotic thrombocytopenic purpura: severe thrombocytopenia (platelets 9 × 10/L), microangiopathic haemolytic anaemia (reticular count 245 × 10/L (20-110)), LDH >5000 U/L (<425)), neurological abnormalities (Glasgow Coma Scale 10/15), renal failure (creatinine 140 µmol/L (<97)), fever (37.7℃). A Disintegrin And Metalloproteinase with a Thrombospondin type 1 motif, member 13 (ADAMTS13) activity of less than 5% and anti-ADAMTS13 antibodies retrospectively confirmed the diagnosis of acquired idiopathic thrombotic thrombocytopenic purpura in pregnancy. The immediate management in the Emergency Department with an interdisciplinary team of Consultant Nephrologists, Intensivists, Haematologists and Obstetricians facilitated prompt diagnosis resulting in immediate plasma exchange (PEX) and coordination of semi-elective delivery of the foetus.
Background: Postpartum hemorrhage is considered a risk factor for pregnancy-associated complement-mediated hemolytic uremic syndrome (CM-HUS; previously known as atypical hemolytic uremic syndrome) but has not been systematically studied.
Objectives: To systematically examine the role of postpartum hemorrhage in precipitating CM-HUS and to describe the characteristics of postpartum hemorrhage-associated CM-HUS, its prognosis and recommended management.
Methods: A systematic review of individual participant data from case series and reports in addition to a case series from our institution.
Background: Immune thrombotic thrombocytopenic purpura (iTTP) is a rare, life-threatening thrombotic microangiopathy. Caplacizumab is the only treatment approved by the European Medicines Agency and the US Food and Drug Administration for iTTP, to be given in combination with plasma exchange therapy (PEX) and immunosuppression (IS). The National Institute for Health and Care Excellence's independent appraisal committee assessed the cost-effectiveness of caplacizumab and concluded that the addition of caplacizumab to PEX+IS is cost-effective under a patient access scheme in the United Kingdom.
Glomerular endothelial cell (GEnC) injury is a common feature across the wide spectrum of glomerular diseases. We recently reported that the endothelial-specific knockout of increases the susceptibility to GEnC injury and subsequent development of subacute thrombotic microangiopathy (TMA). However, the mechanism(s) mediating GEnCs response to injury in TMA are poorly understood.
Background: Primary hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by immune dysregulation. Hematopoietic stem cell transplantation (HSCT) represents the only option for long-term cure for primary HLH. However, only around 25% of patients have a fully HLA-matched donor.
