TTP database

Caratteristiche cliniche e di laboratorio dei pazienti con porpora trombotica trombocitopenica acquisita: quattordici anni del registro TTP di Milano
Ilaria Mancini 1 , Silvia Pontiggia 2 , Roberta Palla 1 , Andrea Artoni 2 , Carla Valsecchi 2 , Barbara Ferrari 2 , Danijela Mikovic 3 , Flora Peyvandi 1 2 ; Italian Group of TTP Investigators

La porpora trombotica trombocitopenica acquisita (TTP) è una rara microangiopatia trombotica causata dal grave deficit immunomediato di ADAMTS13. Riportiamo i dati demografici e correlati alla malattia dei pazienti con TTP acquisita registrati nel Registro TTP di Milano (www.ttpdatabase.org). Abbiamo eseguito uno studio trasversale su 302 individui arruolati nel nostro registro per un episodio acuto di TTP acquisita verificatosi tra il 2002 e il 2015 (femmine 77%; età media all'esordio 40 anni, range interquartile: 30-50). Il 20% dei pazienti presentava concomitanti patologie autoimmuni. Tra i potenziali fattori scatenanti degli episodi acuti, le infezioni erano le più diffuse (27%), seguite dall’uso di estroprogestinici e dalla gravidanza (rispettivamente 5 e 4% delle donne). Alla presentazione, i sintomi sistemici (72%), emorragici (68%) e neurologici (43%) erano i più frequenti, mentre è stata osservata una minore prevalenza di segni e sintomi renali (18%) e cardiovascolari (10%). Quasi tutti gli eventi acuti sono stati trattati con plasmaferesi e steroidi e il 15% con rituximab. L'esacerbazione della TTP acuta si è verificata nel 15% degli eventi. La mortalità correlata al TTP è stata del 5%. Nei sopravvissuti, il numero mediano di procedure di plasmaferesi fino alla remissione è stato 9 (intervallo interquartile: 6-14), più lungo per i primi eventi rispetto alle recidive (differenza mediana 3, intervallo di confidenza al 95%: 2-4). Dei 251 sopravvissuti al primo episodio di TTP con un follow-up di almeno 6 mesi, il 55% ha avuto una ricaduta. In conclusione, la PTT acquisita è una malattia grave con presentazione clinica altamente variabile, che di solito richiede una lunga ospedalizzazione. Il Registro TTP di Milano rappresenta un potente strumento per migliorare la nostra conoscenza e gestione dei TTP acquisiti.

How artificial intelligence might disrupt diagnostics in hematology in the near future
Abstract

Artificial intelligence (AI) is about to make itself indispensable in the health care sector. Examples of successful applications or promising approaches range from the application of pattern recognition software to pre-process and analyze digital medical images, to deep learning algorithms for subtype or disease classification, and digital twin technology and in silico clinical trials. Moreover, machine-learning techniques are used to identify patterns and anomalies in electronic health records and to perform ad-hoc evaluations of gathered data from wearable health tracking devices for deep longitudinal phenotyping. In the last years, substantial progress has been made in automated image classification, reaching even superhuman level in some instances. Despite the increasing awareness of the importance of the genetic context, the diagnosis in hematology is still mainly based on the evaluation of the phenotype. Either by the analysis of microscopic images of cells in cytomorphology or by the analysis of cell populations in bidimensional plots obtained by flow cytometry. Here, AI algorithms not only spot details that might escape the human eye, but might also identify entirely new ways of interpreting these images. With the introduction of high-throughput next-generation sequencing in molecular genetics, the amount of available information is increasing exponentially, priming the field for the application of machine learning approaches. The goal of all the approaches is to allow personalized and informed interventions, to enhance treatment success, to improve the timeliness and accuracy of diagnoses, and to minimize technically induced misclassifications. The potential of AI-based applications is virtually endless but where do we stand in hematology and how far can we go?

https://www.nature.com/articles/s41388-021-01861-y

Spanish registry of thrombotic thrombocytopenic purpura (REPTT): data evidence and new developments
ME Mingot-Castellano, CP Izquierdo, J del Rio Garma - Transfusion and Apheresis …, 2023
Immuno Thrombotic thrombocytopenic purpura (iTTP) is a rare and potentially fatal
disorder characterized by systemic microvascular thrombosis because of a severe
deficiency of ADAMTS13. It is difficult to generate knowledge about TTP because of
read more
https://www.sciencedirect.com/science/article/abs/pii/S147305022300099X

Clinical and Laboratory Features of Patients with Acquired Thrombotic Thrombocytopenic Purpura: Fourteen Years of the Milan TTP Registry
Ilaria Mancini 1 , Silvia Pontiggia 2 , Roberta Palla 1 , Andrea Artoni 2 , Carla Valsecchi 2 , Barbara Ferrari 2 , Danijela Mikovic 3 , Flora Peyvandi 1 2 ; Italian Group of TTP Investigators
Affiliations

PMID: 30861548 DOI: 10.1055/s-0039-1679907

Abstract

Acquired thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy caused by the immune-mediated severe deficiency of ADAMTS13. We hereby report the demographic and disease-related data of acquired TTP patients recorded in the Milan TTP Registry (www.ttpdatabase.org). We performed a cross-sectional study of 302 individuals enrolled in our registry for an acute episode of acquired TTP occurred between 2002 and 2015 (female 77%; median age at onset 40 years, interquartile range: 30-50). Twenty per cent of patients had concomitant autoimmune disorders. Among potential triggers of acute episodes, infections were the most prevalent (27%), followed by estroprogestinics use and pregnancy (5 and 4% of women, respectively). At presentation, systemic (72%), bleeding (68%) and neurological (43%) symptoms were the most frequent, whereas a lower prevalence of renal (18%) and cardiovascular (10%) signs and symptoms was observed. Almost all acute events were treated by plasma exchange and steroids, and 15% by rituximab. Exacerbation of acute TTP occurred in 15% of events. The TTP-related mortality was 5%. In survivors, the median number of plasma exchange procedures to remission was 9 (interquartile range: 6-14), longer for first events than relapses (median difference 3, 95% confidence interval: 2-4). Of 251 survivors of the first TTP episode with at least a 6-month follow-up, 55% had a relapse. In conclusion, acquired TTP is a severe disease with highly variable clinical presentation, usually requiring a long hospitalization. The Milan TTP Registry represents a powerful tool to improve our knowledge and management of acquired TTP.

Complement activation and renal dysfunction in patients with acquired thrombotic thrombocytopenic purpura
…, E Grovetti, L Porcaro, B Ferrari, A Artoni, F Peyvandi - Blood, 2023
Complement activation and renal dysfunction in patients with acquired thrombotic
thrombocytopenic purpura Complement activation and renal dysfunction in patients with
acquired thrombotic thrombocytopenic purpura Blood. 2023 Mar 6;blood.2022018644 …

https://pubmed.ncbi.nlm.nih.gov/36877895/

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…, P Agosti, B Ferrari, R Gualtierotti, A Artoni, F Peyvandi - Research and Practice in …, 2022
Background: Thrombotic thrombocytopenic purpura (TTP) has occasionally been
described after vaccination. Since the availability of anti-SARS-CoV-2 vaccines, 12
cases have been described on a possible association with TTP onset. Aim (s) This …

https://pesquisa.bvsalud.org/global-literature-on-novel-coronavirus-2019...

Involvement of the contact pathway in COVID-19 coagulopathy
Capecchi, M.; Novembrino, C.; Abbattista, M.; Boscolo, M.; Griffini, S.; Grovetti, E.; Artoni, A.; Valenti, L.; Prati, D.; Grasselli, G.; Blasi, F.; Cugno, M.; Peyvandi, F..
Research and Practice in Thrombosis and Haemostasis Conference ; 6(Supplement 1), 2022.
Artigo em Inglês | EMBASE | ID: covidwho-2128125

sky tg24 1 giugno 2020

covid19 e coagulazione

https://www.servizipress.com/DatiWeb/Video/v_0000001758.mp4

Abstract
In acquired thrombotic thrombocytopenic purpura (TTP), an immune-mediated deficiency of the von Willebrand factor–cleaving protease ADAMTS13 allows unrestrained adhesion of von Willebrand factor multimers to platelets and microthrombosis, which result in thrombocytopenia, hemolytic anemia, and tissue ischemia. Caplacizumab, an anti–von Willebrand factor humanized, bivalent variable-domain-only immunoglobulin fragment, inhibits interaction between von Willebrand factor multimers and platelets.

https://www.nejm.org/doi/full/10.1056/NEJMoa1806311?query=featured_home