Presenting Phenotypes and Early Diagnostic Approach in Immune Thrombotic Thrombocytopenic Purpura: A Narrative Review for Emergency and Acute Care Practice

Immune thrombotic thrombocytopenic purpura (iTTP) is a rare condition with multisystem involvement that, in the absence of early recognition and prompt initiation of specific treatment, can be life-threatening. Nowadays, the diagnosis of this pathology represents a challenge in itself, determined by the association of several factors: the heterogeneity of the clinical picture, the frequent absence of all components of the classic pentad, as well as the overlap of neurological, cardiac, abdominal, or renal manifestations with those of much more common medical emergencies. This narrative review synthesizes the current data from the literature on the presentation of iTTP in emergency departments, the clinical and paraclinical elements that outline the clinical picture and support the clinical suspicion, the role of ADAMTS13 activity, and the usefulness of integrating the PLASMIC and French scores in current practice and in therapeutic decision making. The available data highlight the association between thrombocytopenia and microangiopathic hemolytic anemia as a central element of suspicion and support integrating organ involvement assessment, differential diagnosis, and early sampling for ADAMTS13 testing into the evaluation of patients with suspected iTTP. It should also be noted that in patients with high clinical probability of iTTP, the decision to initiate specialized treatment should not be postponed until the ADAMTS13 result is obtained. In this way, a dynamic approach, based on the corroboration of data obtained from the medical history, clinical examination, laboratory and diagnostic investigations, and probability assessment tools, facilitates early diagnosis and rapid access to specific therapy with a favorable impact on the prognosis.