Púrpura trombocitopénica trombótica inmune: sospecha y manejo básico en los servicios de urgencias. Revisión y consenso de un grupo de expertos de las sociedades científicas SEHH y SEMES

Cristina Pascual-Izquierdo1, Pascual Piñera Salmerón2, Francisco Temboury Ruiz3, David Valcárcel Ferreiras4, Sonia Jiménez Hernández5, Ramón Salinas Argente6, Carmen del Arco Galán7, Javier de la Rubia Comos8

Affiliation of the authors

1Hematology and Hemotherapy Hemotherapy, Hospital General Universitario Gregorio Marañón, Madrid. Instituto de Investigación Gregorio Marañón, Member of SEHH. 2Emergency Department, Hospital General General Universitario Reina Sofía, Murcia, Spain. 3Emergency Department, Hospital Universitario Virgen de la Victoria, Málaga, Spain. 4Hematology Service, Vall d’Hebron Institut Oncologic (VHIO), Hospital Vall d’Hebron, Universitat Autònoma de Barcelona, Spain, Member of SEHH. 5Emergency Department, Hospital Clínic, IDIBAPS, Barcelona, Spain. 6Banc de Sang i Teixits, Barcelona, Spain. Member of SEHH. 7Emergency Department, Hospital Universitario La Princesa, Madrid. Research Institute La Princesa, Madrid, Spain. SEMES Scientific Secretary. 8Hematology and Hemotherapy and Hemotherapy, HUP La Fe. Faculty of Medicine, Catholic University of Valencia, Valencia, Spain. Member of SEHH.



Pascual-Izquierdo C, Piñera Salmerón P, Temboury Ruiz F, Valcárcel Ferreiras D, Jiménez Hernández S, Salinas Argente R, et al. 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). Emergencias. 2023;35:44-52


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.


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