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)

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.