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Poster Networking

Platelet Disorders

PB1324 - Brazilian Registry of Thrombotic Thrombocytopenic Purpura: Demographic Analysis and Clinical Profile of an Ambispective Cohort

Tuesday, June 25, 2024
13:45 – 14:45 ICT
Room: Exhibition Hall

    Presenting Author(s)

  • Thaís Dourado Reis Nóbrega, md

    physician
    Hospital das Clínicas da Faculdade de Medicina da USP, São Paulo, São Paulo, Brazil
    São Paulo, Sao Paulo, Brazil

    • Co-Author(s)

  • TB

    Tiago Boechat

    Hematologist
    HEMORIO - Instituto Estadual de Hematologia e Hemoterapia do Rio de Janeiro
    Rio de Janeiro, Rio de Janeiro, Brazil

  • DF

    Denys Fujimoto

    Hematologist
    Santa Casa de São Paulo
    São Pualo, Sao Paulo, Brazil

  • SM

    Sibia Soraya Marcondes

    Hematologist
    Centro de Hemoterapia e Hematologia do Espírito Santo
    Vitoria, Espirito Santo, Brazil

  • SB

    Silvia Bueno

    Hematologist
    Universidade Federal do Paraná
    Curitiba, Parana, Brazil

  • FC

    Fabiana Chagas Camargos Piassi

    Hematologist
    Hospital de Clínicas da Universidade Federal de Minas Gerais
    Belo Horizonte, Minas Gerais, Brazil

  • ST

    Suzanna Tavares

    Hematologist
    HEMOCE - Centro de Hematologia e Hemoterapia do Ceará
    Fortaleza, Ceara, Brazil

  • Alessandra Nunes Loureiro Prezotti, MD, MsC, PhD

    Hematologist
    HEMOES - Centro de Hemoterapia e Hematologia do Espírito Santo
    Vitória, Espirito Santo, Brazil

  • JD

    Juliana Souza Mario Duarte

    Hematologist
    HEMOES - Centro de Hemoterapia e Hematologia do Espírito Santo
    Vitória, Espirito Santo, Brazil

  • LC

    Lucas Césped

    medical student
    Centro de Hematologia e Hemoterapia da Unicamp
    Unicamp, Sao Paulo, Brazil

  • EO

    Erica Okazaki

    MD
    Department of Hematology, Hemotherapy and Cellular Therapy, Hospital das Clínicas of University of São Paulo Medical School (HCFMUSP), São Paulo, Brazil
    São Paulo, Sao Paulo, Brazil

  • BS

    Bianca Stefanello

    MD
    Department of Hematology, Hemotherapy and Cellular Therapy, Hospital das Clínicas of University of São Paulo Medical School (HCFMUSP), São Paulo, Brazil
    São Paulo, Sao Paulo, Brazil

  • Carolina Costa-Lima

    Hematologist
    Hemocentro UNICAMP, University of Campinas, Campinas, SP, Brazil
    Campinas, Sao Paulo, Brazil

  • PV

    Paula Villaça

    Hematologist
    Hospital das Clínicas da Faculdade de Medicina da USP, São Paulo, São Paulo, Brazil
    São Paulo, Sao Paulo, Brazil

  • FO

    Fernanda Andrade Orsi

    MD
    Department of Hematology, Hemotherapy and Cellular Therapy, Hospital das Clínicas of University of São Paulo Medical School (HCFMUSP), São Paulo, Brazil
    São Paulo, Sao Paulo, Brazil

Background: Acquired Thrombotic Thrombocytopenic Purpura (aTTP) carries a high-morbidity and mortality burden. Accurate diagnosis and treatment are crucial; however, existing data come from North American and European registries, lacking information from Latin America.

Aims: To investigate the epidemiological and clinical profile of aTTP in Brazil.

Methods: Multicenter ambispective study that analyzed clinical manifestations, treatment response and prognosis of Brazilian aTTP cohort between May-2018 and December-2023. TTP was diagnosed when PLASMIC score was ≥ 6 or ADAMTS13 levels < 10%.

Results: A total of 68 cases from 8 centers were included. The median age was 37 (IQR 27-46), with 77% women, 8% having experienced prior TTP episodes. Upon admission, 88% presented with bleeding, and 66% had severe neurological symptoms, which had initiated 8 days (IQR 3.8-15) and 3 days (IQR 1-7.8) prior to admission, respectively. ADAMTS13 was available in 39 cases (59.1%). Treatment comprised plasma-exchange (89.7%), corticosteroids (94.1%), rituximab (50%), and caplacizumab (4.4%). Median plasma-exchange duration was 7 days (IQR 5-13.5), hospital stay was 13 days (IQR 8-28). Initially, 23% were refractory, 20% had TTP exacerbation and 16.2% died. Hemodynamic instability at admission (OR 6.75; 95%CI 1.15-39.40, P=0.003) was independently associated with the risk of death. At 12-month follow-up, we observed 9 (13%) recurrences, 9 neurological (chronic headache), 3 cardiovascular and 2 psychiatric sequelae. Rituximab use in the acute phase prevented recurrence in the subsequent 6 (P=0.02) and 12 months (P=0.04).

Conclusion(s): Gender and age distribution were similar to other cohorts (French Registry, Oklahoma), indicating consistency across diverse populations. Nevertheless, differences were observed, including delayed diagnosis, a high prevalence of severe neurological symptoms and cardiovascular collapse at diagnosis, prolonged time to platelet recovery, extended in-hospital stay, and high mortality at acute presentation. These findings suggest that in developing nations, aTTP diagnosis and treatment may lag behind developed countries, underscoring the need for tailored healthcare approaches.