Immune thrombotic thrombocytopenic purpura: clinical suspicion and basic management in emergency departments - an expert review and consensus statement from the Spanish societies of hematology and hemotherapy (SEHH) and emergency medicine (SEMES).

Autor: Pascual-Izquierdo C; Servicio de Hematología y Hemoterapia, Hospital General Universitario Gregorio Marañón, Madrid. Instituto de Investigación Gregorio Marañón, Miembro de SEHH., Piñera Salmerón P; Servicio de Urgencias, Hospital General Universitario Reina Sofía, Murcia, España., Temboury Ruiz F; Servicio de Urgencias, Hospital Universitario Virgen de la Victoria, Málaga, España., Valcárcel Ferreiras D; Servicio de Hematología, Vall d'Hebron Institut Oncologic (VHIO), Hospital Vall d'Hebron, Universitat Autònoma de Barcelona, España, Miembro de SEHH., Jiménez Hernández S; Área de Urgencias, Hospital Clínic, IDIBAPS, Barcelona, España., Salinas Argente R; Banc de Sang i Teixits, Barcelona, España. Miembro de SEHH., Del Arco Galán C; Servicio de Urgencias, Hospital Universitario La Princesa, Madrid. Instituto Investigación La Princesa, Madrid, España. Secretaria Científica SEMES., de la Rubia Comos J; Servicio de Hematología y Hemoterapia, HUP La Fe. Facultad de Medicina, Universidad Católica de Valencia, Valencia. Miembro de SEHH.
Jazyk: English; Spanish; Castilian
Zdroj: Emergencias : revista de la Sociedad Espanola de Medicina de Emergencias [Emergencias] 2023 Feb; Vol. 35 (1), pp. 44-52.
DOI: 10.55633/s3me/E098.2023
Abstrakt: Text: Acquired or immune thrombotic thrombocytopenic purpura (TTP) are thrombotic microangiopathies associated with high mortality if treatment is not started early. Onset is usually sudden, meaning that the condition is often diagnosed in hospital emergency departments, where TTP must be suspected as early as possible. These guidelines were drafted by specialists in emergency medicine and hematology to cover the diagnosis, referral, and treatment of patients suspected of immune-mediated TTP who require emergency care. Immune TTP should be suspected whenever a patient presents with hemolytic microangiopathy and has a negative Coombs test, and thrombocytopenia, possibly in conjunction with fever and neurologic and cardiac alterations. If one of the existing diagnostic algorithms indicates there is a high probability that the patient has immune TTP, plasma exchange therapy should be started along with immunosuppressants. Treatment with caplacizumab should also be considered. The patient should be referred immediately to the hematology department within the same hospital or a referral hospital.
Databáze: MEDLINE