Cognitive decline in thrombotic thrombocytopenic purpura survivors: The role of white matter health as assessed by MRI.

Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare condition caused by severe ADAMTS13 deficiency, leading to platelet aggregation and thrombosis. Despite treatment, patients are prone to cognitive impairment and depression. We investigated brain changes in iTTP patients during remission using advanced magnetic resonance imaging (MRI) techniques, correlating these changes with mood and neurocognitive tests. Twenty iTTP patients in remission (30 days post-haematological remission) were compared with six healthy controls. MRI scans, including standard and specialized sequences, were conducted to assess white matter health. Increased T1 relaxation times were found in the cingulate cortex (p < 0.05), and elevated T2 relaxation times were observed in the cingulate cortex, frontal, parietal and temporal lobes (p < 0.05). Pathological changes in these areas are correlated with impaired cognitive and depressive scores in concentration, short-term memory and verbal memory. This study highlights persistent white matter damage in iTTP patients, potentially contributing to depression and cognitive impairment. Key regions affected include the frontal lobe and cingulate cortex. These findings have significant implications for the acute and long-term management of iTTP, suggesting a need for re-evaluation of treatment approaches during both active phases and remission. Further research is warranted to enhance our understanding of these complexities.

Consensus on the standardization of terminology in thrombotic thrombocytopenic purpura and related thrombotic microangiopathies.

Essentials An international collaboration provides a consensus for clinical definitions. This concerns thrombotic microangiopathies and thrombotic thrombocytopenic purpura (TTP). The consensus defines diagnosis, disease monitoring and response to treatment. Requirements for ADAMTS-13 are given. SUMMARY: Background Thrombotic thrombocytopenic purpura (TTP) and hemolytic-uremic syndrome (HUS) are two important acute conditions to diagnose. Thrombotic microangiopathy (TMA) is a broad pathophysiologic process that leads to microangiopathic hemolytic anemia and thrombocytopenia, and involves capillary and small-vessel platelet aggregates. The most common cause is disseminated intravascular coagulation, which may be differentiated by abnormal coagulation. Clinically, a number of conditions present with microangiopathic hemolytic anemia and thrombocytopenia, including cancer, infection, transplantation, drug use, autoimmune disease, and pre-eclampsia and hemolysis, elevated liver enzymes and low platelet count syndrome in pregnancy. Despite overlapping clinical presentations, TTP and HUS have distinct pathophysiologies and treatment pathways. Objectives To present a consensus document from an International Working Group on TTP and associated thrombotic microangiopathies (TMAs). Methods The International Working Group has proposed definitions and terminology based on published information and consensus-based recommendations. Conclusion The consensus aims to aid clinical decisions, but also future studies and trials, utilizing standardized definitions. It presents a classification of the causes of TMA, and criteria for clinical response, remission and relapse of congenital and immune-mediated TTP.

Compliance with a massive transfusion protocol (MTP) impacts patient outcome.

BACKGROUND: About 5% of civilian trauma requires massive transfusion. Protocolized resuscitation with blood products to achieve high plasma:RBC ratio has been advocated to improve survival. Our objectives were to measure compliance to our institutional MTP, to identify quality assurance activities that could improve protocol compliance and to determine if protocol compliance was related to patient outcome. METHODS: The investigators determined 13 compliance criteria based upon our institutional protocol. We measured compliance in 72 consecutive MTP activations between January 2010 and September 2011 at a Level I trauma centre. Data elements were retrospectively retrieved from blood bank, trauma registry and clinical records. Patients were stratified into three groups based on compliance level, and mortality differences were compared. RESULTS: Average compliance for the cohort (n=72) was 66%. The most common cause of non-compliance was failure to send a complete haemorrhage panel from the trauma bay (96%). Failure to monitoring blood work every 30min occurred in 89% of cases. Delay in activation and deactivation occurred in 50% and 50% respectively. Non-compliance to protocol-based administration of blood products happened in 47%. The cohort was stratified into three groups based on compliance, A: <60%, B: 60-80% and C: >80% (low, moderate and high compliance groups). There was no statistical significance with regard to median age, median ISS, ED SBP, ED GCS and AIS of the head/spine, chest and abdomen. The mortality rates in each group were 62%, 50% and 10% in the low, moderate and high compliance groups respectively. Mortality differences were compared using adjusted logistic regression. The OR for mortality between Groups A and B=1.1 [95% CI 0.258-4.687 (P=0.899)] while the OR for mortality between Groups C and B=0.02 [95% CI <0.001-0.855 (P=0.041)]. CONCLUSIONS: Measures should be directed towards provider and system factors to improve compliance. In this study, there was an association between survival and higher level of compliance.