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Onkológia S1/2024
Thrombotic thrombocytopeic purpura – urgent situation in haematology
Acquired thrombotic thrombocytopeic purpura is an acute life-threatening disorder, associated with a mortality of 90% if unrecognised and untreated. Is characterized by microangiopathic hemolytic anemia, severe thrombocytopenia and multiorgan failure due to autoimmune-mediated deficiency in ADAMTS13 activity. Rapid recognition of TTP is crucial to initiate appropriate treatment. Standard of care currently includes plasma exchange, including glucocorticoids and rituximab. Caplacizumab is a novel agent approved for treatment adult patients with aTTP.
Keywords: acquired thrombotic thrombocytopeic purpura, ADAMTS13 deficiciency, plasma exchange, caplacizumab
