From the Research
Thrombotic thrombocytopenic purpura (TTP) typically presents with a pentad of symptoms: thrombocytopenia, microangiopathic hemolytic anemia, neurological abnormalities, renal dysfunction, and fever, although not all five features need to be present for diagnosis. The most critical aspect of TTP presentation is the timely recognition of its symptoms to initiate prompt treatment. According to the most recent and highest quality study 1, TTP is characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and ischemic end-organ injury due to microvascular platelet-rich thrombi, resulting from a severe deficiency of the von Willebrand factor (VWF)-cleaving protease, ADAMTS13.
Key Features of TTP Presentation
- Thrombocytopenia
- Microangiopathic hemolytic anemia
- Neurological abnormalities
- Renal dysfunction
- Fever
Diagnostic Approach
Diagnosis is based on the presence of clinical symptoms, laboratory aberrations consistent with microangiopathic hemolytic anemia, decreased ADAMTS13 activity, and possibly the presence of anti-ADAMTS13 autoantibodies 2. Laboratory findings typically show severe thrombocytopenia, hemolytic anemia with schistocytes on peripheral smear, elevated LDH, low haptoglobin, and negative direct Coombs test.
Management
The most critical initial management for TTP is prompt plasma exchange (PEX) therapy, which should be initiated within 4-8 hours of suspected diagnosis, along with corticosteroids. Caplacizumab should be added for acquired TTP, and rituximab is recommended for refractory cases or as upfront therapy in some protocols 1. It is essential to consider TTP as a differential diagnosis in patients presenting with thrombocytopenia and microangiopathic hemolytic anemia without an obvious cause, as timely diagnosis and treatment are critical for outcome 3.