Deadly interaction between acquired thrombotic thrombocytopenic purpura and posterior reversible encephalopathy syndrome: a case study report
A recent case study highlights the diagnostic challenges associated with coexisting Thrombotic Thrombocytopenic Purpura (TTP) and Posterior Reversible Encephalopathy Syndrome (PRES). The case involved a 22-year-old male who presented with a series of severe symptoms, including vomiting, headache, right upper limb weakness, hypertensive crisis, and generalised seizures.
Upon admission, laboratory investigations revealed anaemia, thrombocytopenia, acute kidney injury, and signs of hemolysis. An MRI revealed hyperintense lesions involving the bilateral occipito-parieto-frontal areas, cerebellar hemispheres, pons, and right lentiform nucleus, suggesting PRES. However, the CT scan did not show any acute intracranial abnormalities.
TTP, a rare but life-threatening thrombotic microangiopathy, was diagnosed due to the patient's severe thrombocytopenia, microangiopathic hemolytic anemia, and multiorgan dysfunction. The patient had schistocytes and markedly reduced ADAMTS13 activity, leading to a diagnosis of acquired TTP.
The coexistence of TTP and PRES presents a diagnostic challenge due to the overlapping clinical features of thrombotic microangiopathies and neurological syndromes. Rapid recognition and initiation of plasma exchange, alongside supportive care for PRES, offer the best outcomes. 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. On the second day of hospitalization, the patient developed two episodes of generalised tonic-clonic seizures, further complicating the diagnosis and treatment.
Acquired TTP is the predominant form and is frequently associated with autoimmune diseases, malignancies, pregnancy, infections, and certain medications. MRI findings confirmed the presence of PRES. Distinguishing TTP from other thrombotic microangiopathies with renal involvement is crucial to avoid treatments that may worsen outcomes, such as certain plasma therapies in non-TTP conditions.
Timely, coordinated multidisciplinary management involving hematology, neurology, and critical care improves prognosis. 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. This case underscores the importance of early and accurate diagnosis and rapid intervention in managing coexisting TTP and PRES to improve patient outcomes.
References:
[1] Banerjee, S., & Rizzo, A. (2018). Thrombotic Thrombocytopenic Purpura. In StatPearls [Internet]. StatPearls Publishing.
[3] Gulati, A., & Rizzo, A. (2020). Posterior Reversible Encephalopathy Syndrome. In StatPearls [Internet]. StatPearls Publishing.
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