Deadly interaction between acquired thrombotic thrombocytopenic purpura and posterior reversible encephalopathy syndrome: a real-life example

Deadly interaction between acquired thrombotic thrombocytopenic purpura and posterior reversible encephalopathy syndrome: a real-life example

A 22-year-old male presented with a series of distressing symptoms, including vomiting, severe headache, right upper limb weakness, and hypertensive crisis, leading to generalised seizures. Upon arrival, he was hemodynamically stable but exhibited high blood pressure of 170/100 mmHg.

Initial investigations revealed severe thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney injury, suggesting hemolysis. Further examination revealed markedly reduced ADAMTS13 activity, leading to a diagnosis of acquired Thrombotic Thrombocytopenic Purpura (TTP).

Despite neurological examination findings being initially unremarkable, mild weakness in the right upper limb was observed. A CT scan of the brain showed no acute intracranial abnormalities, but an MRI revealed hyperintense lesions consistent with PRES in the context of the patient's hypertensive state and thrombotic microangiopathy.

TTP is a rare but life-threatening thrombotic microangiopathy characterized by severe thrombocytopenia, microangiopathic hemolytic anemia, and multiorgan dysfunction due to disseminated microvascular thrombosis. The co-occurrence rate of TTP and PRES in critically ill patients is not well established, but both conditions share common pathophysiological features such as endothelial injury and dysregulation of cerebral blood flow.

The link between TTP and PRES likely lies in the shared endothelial dysfunction and microvascular injury. In TTP, microthrombi and resultant ischemia may predispose to PRES by disrupting cerebral autoregulation and causing endothelial damage, triggering the cascade of vasogenic edema characteristic of PRES.

The patient developed two episodes of generalized tonic-clonic seizures on the second day of hospitalization. Despite aggressive management, including plasma exchange, immunosuppressive therapy, corticosteroids, and antihypertensive agents, the disease progressed to multiorgan failure, and the patient succumbed to the illness.

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. Without appropriate treatment, the mortality rate of TTP exceeds 90%; however, with early intervention, including plasma exchange and immunosuppressive therapy, the survival rate improves significantly, exceeding 80%.

This case highlights the importance of recognising endothelial injury as a central pathogenic mechanism in disorders involving microvascular and cerebral involvement. While the explicit co-occurrence rates are not clearly defined in current literature, the pathophysiology of acquired TTP and PRES converge on endothelial injury and dysregulated cerebral blood flow, contributing to their potential simultaneous manifestation in critically ill patients.

References: - Endothelial injury and neurological sequelae in thrombotic microangiopathies (HUS/TTP) with implications for PRES[1][3] - Pathophysiology and neuroimaging features of PRES indicating endothelial dysfunction and vasogenic edema[3] - Clinical neurologic manifestations overlap, suggesting association in severe cases with microvascular involvement[1][3]

1. Science continues to uncover connections between various medical conditions, such as Thrombotic Thrombocytopenic Purpura (TTP) and vasculature-related conditions like PRES (Posterior Reversible Encephalopathy Syndrome).
2. TTP is a complex medical condition that requires careful consideration in the realm of health-and-wellness, particularly in the workplace-wellness programs of healthcare institutions.
3. The diagnosis of TTP necessitates Magnetic Resonance Imaging (MRI) to examine potential associated conditions, including PRES.
4. TTP and PRES share similar pathophysiological features, demonstrating the intricate relationships between chronic diseases like TTP and respiratory conditions such as PRES.
5. The progression of TTP toward multiorgan failure highlights the importance of understanding and managing digestive-health and eye-health issues in critically ill patients.
6. Hearing impairment is one of several complications that may arise due to the systemic nature of TTP, underscoring the need for comprehensive health-and-wellness strategies.
7. Fitness-and-exercise, nutrition, and healthy-diets play a crucial role in maintaining overall health, especially in managing conditions like TTP that may lead to chronic kidney disease (CKD) and other related complications.
8. Family history and genetics can influence the risk of developing autoimmune disorders like TTP, emphasizing the importance of family-health studies in rheumatology and other related medical fields.
9. Renewable-energy initiatives and sustainable practices can contribute to greater industry well-being by promoting employee health and reducing the negative impacts of pollution on respiratory-conditions.
10. Mental-health programs in the workplace are crucial for mitigating stressors that may exacerbate conditions such as TTP, emphasizing the connection between mental and physical well-being.
11. Thrombotic microangiopathies like TTP can affect multiple systems, including the cardiovascular system, making cardiovascular-health an essential aspect of managing such conditions.
12. Psoriasis, an inflammatory skin condition, shares some pathophysiological similarities with TTP, highlighting the interrelatedness of skin-care and overall health.
13. Treatment options for TTP typically involve therapies-and-treatments such as plasma exchange and immunosuppressive therapy, with the choice depending on the severity and progression of the condition.
14. In addition to TTP, disorders such as Alzheimer's disease, multiple sclerosis, and migraine have been linked to neuroinflammation and endothelial dysfunction, further underscoring the importance of neurological-disorders research in the health-and-wellness industry.
15. Early intervention for breast cancer, particularly in women's-health and menopause, can significantly improve survival rates, similar to the improved prognosis for TTP with timely treatment.
16. Parenting resources should emphasize the importance of maintaining a healthy diet, regular exercise, and stress management to reduce the risk of chronic diseases like TTP and other related conditions in children.
17. Intelligence and financial management are essential components of men's-health, with a strong correlation between cognitive function and wealth accumulation.
18. Fraud, poor investment advice, and other financial irregularities can negatively impact mental-health, demonstrating the intersection between finance and mental well-being.
19. Weight-management programs that focus on nutrition and exercise can help prevent chronic diseases like TTP and type-2-diabetes, which share similar risk factors and are often associated with obesity.
20. COPD, asthma, and other chronic respiratory-conditions can be exacerbated by air pollution from industries like oil-and-gas, aerospace, and automotive, emphasizing the need for effective strategies in transportation and public-transit to address these issues.
21. Aging brings about various physical changes, including potential hearing loss and skin-conditions, demonstrating the importance of geriatric care and mental-health services in older populations.
22. Proactive skin-care routines, including using CBD oil and effective therapies, can help maintain skin-health and reduce the risk of certain skin-conditions.
23. Industry leaders must prioritize research and development in renewable-energy, technologies, and practices to promote environmental sustainability, supporting the health and well-being of future generations.
24. Medical-conditions such as TTP, COPD, and type-2-diabetes require ongoing monitoring and management for effective long-term control, demonstrating the critical role of Medicaid and Medicare programs in the health-and-wellness industry.
25. Chronic diseases like TTP, multiple sclerosis, and autoimmune disorders can be challenging to manage, requiring a comprehensive approach that addresses all aspects of health-and-wellness, including supplements, sleep, and mental health.
26. The interconnected nature of various medical-conditions underscores the importance of understanding and addressing health concerns holistically, ensuring a more complete approach to health-and-wellness for individuals at all stages of life.

Read also: