From the Research
Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder that requires immediate medical attention, and the most effective treatment involves daily plasma exchange therapy (plasmapheresis) until platelet counts normalize, as supported by the most recent study 1.
Treatment Overview
The treatment of TTP typically involves:
- Plasma exchange therapy (plasmapheresis) daily until platelet counts normalize
- Corticosteroids such as methylprednisolone (1mg/kg/day) or prednisone (1mg/kg/day) may be added
- For refractory cases, rituximab (375mg/m² weekly for 4 weeks) may be added
- Supportive care includes red blood cell transfusions as needed, though platelet transfusions are generally avoided unless there is life-threatening bleeding
Pathophysiology and Diagnosis
TTP is caused by a deficiency in ADAMTS13, an enzyme that breaks down von Willebrand factor, leading to excessive platelet clumping in small blood vessels. This results in the classic pentad of symptoms:
- Thrombocytopenia
- Microangiopathic hemolytic anemia
- Neurological abnormalities
- Kidney dysfunction
- Fever A diagnosis of TTP can be made in patients with thrombocytopenia and microangiopathic hemolytic anemia without an alternate cause, as noted in 2.
Importance of Prompt Treatment
Without prompt treatment, TTP has a high mortality rate, but with appropriate therapy, survival rates exceed 80%, as seen in 3. The critical determinant of outcome is timely diagnosis and treatment, emphasizing the need for immediate plasma exchange and adjuvant therapies as necessary.
Key Considerations
- Plasma exchange should be initiated as soon as possible and repeated daily until platelet counts stabilize
- The use of corticosteroids and rituximab may be beneficial in certain cases, as discussed in 4
- Supportive care, including red blood cell transfusions, is crucial in managing the condition
- The emergency physician should be aware of TTP's range of presentations, diagnostic criteria, and treatment options to ensure timely and effective management, as highlighted in 2.