From the Guidelines
Thrombotic thrombocytopenic purpura (TTP) is characterized by a pentad of clinical signs and symptoms, including microangiopathic hemolytic anemia, thrombocytopenia, neurological abnormalities, renal dysfunction, and fever, as described in the study by 1. The clinical presentation of TTP can vary, but common signs and symptoms include:
- Microangiopathic hemolytic anemia, which can lead to jaundice, pallor, and dark urine
- Thrombocytopenia, which can cause purpura, petechiae, and bleeding from mucous membranes, gums, or gastrointestinal tract
- Neurological abnormalities, ranging from headaches, confusion, and altered mental status to seizures, focal deficits, and coma
- Renal dysfunction, which can present as hematuria, proteinuria, or acute kidney injury
- Fever, fatigue, weakness, and abdominal pain
Key Laboratory Findings
The diagnosis of TTP is supported by laboratory findings, including:
- Severe thrombocytopenia (usually <30,000/μL)
- Hemolytic anemia with schistocytes (fragmented red blood cells) on peripheral blood smear
- Elevated lactate dehydrogenase (LDH)
- Decreased haptoglobin
- Negative direct Coombs test As noted in the study by 1, TTP is a medical emergency that requires prompt recognition and treatment to prevent potentially fatal outcomes.
From the Research
Clinical Signs and Symptoms of TTP
The clinical signs and symptoms of Thrombotic Thrombocytopenic Purpura (TTP) include:
- Thrombocytopenia
- Microangiopathic hemolytic anemia
- Neurological abnormalities, such as hallucinations, bizarre behavior, altered mental status, headache, and neurological changes including fluctuating mental status 2, 3
- Renal dysfunction 2, 3
- Fever 2, 3
- Skin reactions, such as purpura and ecchymoses 3
Organ Ischemia and Microvascular Thrombi
TTP is characterized by organ ischemia linked to disseminated microvascular platelet-rich thrombi 4, 5. This can lead to a range of clinical symptoms, including:
- Neurological symptoms, such as headache and altered mental status
- Renal dysfunction
- Cardiac symptoms
Diagnosis and Treatment
Diagnosis of TTP is based on the presence of thrombocytopenia, microangiopathic hemolytic anemia, and organ ischemia 4, 5. Treatment typically involves daily plasma exchange with fresh frozen plasma replacement and immunosuppression with corticosteroids 2, 4, 5. In some cases, additional therapies, such as rituximab or caplacizumab, may be used 4, 5.