Thrombotic thrombocytopenic purpura: a hematological emergency

Thrombotic thrombocytopenic purpura (TTP) is a rare but severe disease characterized by mechanical hemolytic anemia and consumptive thrombocytopenia leading to disseminated microvascular thrombosis that causes signs and symptoms of organ ischemia and functional damage. TTP is diagnosed by the presence of microangiopathic hemolytic anemia and thrombocytopenia in a patient who frequently presents with central nervous system involvement and, to a lesser extent, renal dysfunction.

We present the case of a 23-year-old female with TTP, diagnosed by the presence of the neurological symptoms, microangiopathic hemolytic anemia and severe thrombocytopenia (platelets 4000/μL). During the clinical evolution, the patient presented the hepatic cytolysis syndrome, following disseminated microvascular thrombosis inside the liver, representing an atypical damage. The diagnosis was difficult because it was necessary to make differential diagnosis with other diseases that evolve with microangiopathic hemolytic anemia in a short time to be able to initiate plasmapheresis. Initiation of the plasmapheresis as soon as possible was the goal of our treatment. Following the plasmapheresis combined with administration of corticosteroids was achieved complete resolution of all symptoms.

TTP is a hematological emergency and diagnostic challenge. The critical determinant of outcome is timely diagnosis and treatment. Once the diagnosis is suspected, life-saving therapeutic plasma exchange therapy is initiated.