Deadly Interaction between Acquired Thrombotic Thrombocytopenic Purpura and Posterior Reversible Encephalopathy Syndrome: A Real-Life Example
A 22-year-old male was recently admitted to the hospital, presenting with symptoms including vomiting, headache, right upper limb weakness, hypertensive crisis, and generalized seizures. Initial investigations revealed severe thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney injury.
The MRI findings did not show any acute intracranial abnormalities in the initial CT scan. However, an MRI revealed hyperintense lesions suggestive of PRES. The EEG revealed a diffuse spike-and-wave pattern consistent with generalized seizure activity, further complicating the case.
The patient's condition led to the diagnosis of acquired TTP due to markedly reduced ADAMTS13 activity and the presence of schistocytes. Laboratory investigations also revealed anaemia, thrombocytopenia, elevated LDH levels, and other indicators of hemolysis.
The patient was treated with plasma exchange, immunosuppressive therapy, corticosteroids, and antihypertensive agents. Despite aggressive management, the disease progressed to multiorgan failure, and the patient succumbed to the illness.
TTP is a rare but life-threatening thrombotic microangiopathy caused by severe ADAMTS13 deficiency. Acquired TTP is the predominant form and is frequently associated with autoimmune diseases, malignancies, pregnancy, infections, and certain medications. PRES is a neurological disorder usually linked to endothelial dysfunction and vasogenic edema, often reversible with treatment.
When TTP and PRES coexist, clinical reports suggest a potentially more severe clinical course. TTP with neurological manifestations including PRES may indicate widespread microvascular injury and increased risk for adverse outcomes such as seizures, stroke, or death. Early recognition and aggressive treatment are crucial for improving prognosis.
The exact frequency of their co-occurrence is not well established in general critical care literature due to the rarity and complexity of each condition. However, when they do occur together, the co-occurrence signals a serious condition requiring prompt intervention.
Without appropriate treatment, the mortality rate of TTP exceeds 90%, but with early intervention, the survival rate improves significantly, exceeding 80%. Comprehensive epidemiological or prognostic studies quantifying the impact of co-occurrence in critically ill patients appear lacking in the recent literature.
The incidence of acquired TTP is estimated to be 3-6 cases per million per year, with a higher prevalence among females and young adults. Despite this, the search results do not provide direct data on the co-occurrence rate of TTP and PRES in critically ill patients, nor the specific prognostic impact of their simultaneous presence.
In conclusion, the co-occurrence of TTP and PRES is a serious condition that requires prompt intervention. Early recognition and aggressive treatment are crucial for improving prognosis, although comprehensive studies quantifying the impact of co-occurrence in critically ill patients are lacking.
- The MRI scan, a crucial tool in medical-conditions diagnosis, revealed hyperintense lesions indicative of PRES, which is a neurological disorder often associated with chronic diseases like TTP.
- Science has determined that TTP, a life-threatening thrombotic microangiopathy, is frequently linked to malignancies, and when it coexists with PRES, the clinical course can be more severe, potentially leading to increased risk for adverse outcomes such as seizures, stroke, or death.
- In the context of health-and-wellness, early recognition and aggressive treatment of TTP and PRES co-occurrence are crucial for improving prognosis, as comprehensive studies have shown that without appropriate intervention, the mortality rate of TTP is over 90%, but with early intervention, survival rates can exceed 80%.
- Mental-health and neurological-disorders alike require immediate attention when TTP and PRES coexist, as their co-occurrence is a serious condition that signals a need for prompt intervention, particularly in critically ill patients, although specific prognostic data remains limited due to the rarity and complexity of each condition.
