Cerebral Infarction in Immune Thrombotic Thrombocytopenic Purpura Is Associated with Old Age, Hypertension, Smoking, and Anti-ADAMTS13 Ig, But Not with Mortality

Abstract

Background Neurological involvement is common in patients with immune thrombotic thrombocytopenic purpura (iTTP), but the frequency, risk factors, and outcomes of these with imaging-confirmed stroke in iTTP are not known. Methods We selected 66 out of 109 iTTP patients with neurological signs and symptoms and reviewed their CT/MRI (computed tomography/magnetic resonance imaging) findings for the evidence of stroke and other clinical information in Alabama TTP Registry. Results Of these, 52 (78.8%) had their CT/MRI done on admission in whom 22 (42.3%) were positive for multiple acute or chronic infarcts. The patients with image-confirmed ischemic stroke were older, and appeared to be associated with a history of hypertension and smoking. Additionally, patients with imaging-confirmed stroke showed higher plasma concentrations of anti-ADAMTS13 IgG than those without stroke. More interestingly, there was no statistically significant difference in the rate of exacerbation and 60-day mortality between those with and without stroke. Conclusion Ischemic cerebral infarcts are common findings in brain imaging studies of patients with acute iTTP; old age, chronic hypertension, and smoking, as well as high plasma concentrations of anti-ADAMTS13 IgG may be the potential risk factors for cerebral infarction in these patients. The presence of image-confirmed ischemic stroke, however, does not predict exacerbation and 60-day mortality, although the long-term effect of such ischemic brain damage on cognitive function and quality of life remains to be determined.

The standard of care for immune thrombotic thrombocytopenic purpura today

“…Patients may present initially with some nonspecific symptoms including fatigue, headache, fever, pale skin, and rashes; some patients may also experience abdominal pain, extremity weakness, and symptoms of respiratory and urinary infections. In more severe cases, signs and symptoms of ischemic stroke, 22,23 myocardial infarction, 24,25 seizure, and coma 26,27 may be observed in the emergency department.…”

“…14,17,28 A computerized tomography (CT)/magnetic resonance imaging (MRI) scan may reveal large and/or small infarctions in the brain of patients with acute iTTP. 22,33,34 Based on these clinical and laboratory data, a presumptive diagnosis of iTTP can be made.…”

“…Approximately 50% of patients with acute iTTP may have elevated levels of serum or plasma troponins 14,30‐32 and mild to moderate elevation of serum creatine 14,17,28 . A computerized tomography (CT)/magnetic resonance imaging (MRI) scan may reveal large and/or small infarctions in the brain of patients with acute iTTP 22,33,34 . Based on these clinical and laboratory data, a presumptive diagnosis of iTTP can be made.…”

“…Prior to caplacizumab, 10% to 20% of patients died and nearly 50% of patients experienced exacerbations or refreactoriness despite daily TPE, steroids, and rituximab 14,24 . Many patients who survive the acute episode may suffer from cognitive decline and depression, 78 as well as ischemic stroke 22,79 . Thus, it is important to stop microvascular thrombosis with caplacizumab as early as possible, while trying to address the underlying autoantibody‐mediated inhibition of ADAMTS‐13 activity with immunosuppressives, which may take weeks to months.…”

Two ischemic stroke events within 48 h: a case report of an unusual presentation of thrombotic thrombocytopenic purpura

“…However, TTP considers a rare cause of ischemic stroke [ 15 ], although there are similar cases in the literature of patients being diagnosed with TTP after a cerebrovascular accident [ 9 , 21 , 24 – 26 ]. A recent study on patients with TTP who presented with neurological symptoms suggested that old age, hypertension, smoking, and high plasma concentrations of anti-ADAMTS-13 IgG may be the risk factors for developing cerebral infarction in these patients [ 27 ]. Furthermore, they reported no association between iTTP stroke and diabetes, however, our patient was diabetic [ 27 ].…”