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Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder caused by a congenital or acquired deficiency of the ADAMTS13 enzyme.
When ADAMTS13 activity is reduced, ultra-large von Willebrand factor (vWF) multimers accumulate in the circulation and are not adequately cleaved. This leads to pathological platelet aggregation and the formation of widespread microvascular thrombi. As a consequence, platelet consumption and microangiopathic hemolytic anemia develop.
Classical clinical pentad:
fever, thrombocytopenia, hemolytic anemia, neurological symptoms, and renal dysfunction.
However, the complete pentad is now observed infrequently; the disease may present with only part of these features.
Diagnosis is based on the combination of clinical findings and laboratory evidence of microangiopathic hemolytic anemia (presence of schistocytes on peripheral smear, elevated LDH, reduced haptoglobin), along with severely decreased ADAMTS13 activity.
Treatment requires urgent therapeutic plasma exchange (plasmapheresis) and immunosuppressive therapy.
Without prompt intervention, the condition is associated with a very high mortality rate, while early treatment dramatically improves outcomes.
