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Cause And Symptoms

Complications

Diagnosis And Treatment

Overview

Prevention

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Scientific Content

<h2><span style="color:#6f1813;">What is TTP?</span></h2>
<p><strong>Thrombotic thrombocytopenic purpura (TTP)— A rare, life-threatening medical emergency</strong></p>
<p>TTP is a rare, life-threatening thrombotic microangiopathy (TMA). There are approximately 3 cases per million people.<sup>1,2</sup><br>
  The name thrombotic thrombocytopenic purpura describes the 3 main characteristics of the disease.</p>

<p><strong>Thrombotic:</strong>&nbsp;characterized by the formation of microthrombi (blood clots) within a blood vessel, which clog the vessel and prevent blood circulation.</p>

<p><strong>Thrombocytopenic:</strong>&nbsp;a lower-than-normal platelet count due to the consumption of platelets during microthrombi formation, which causes occlusion of microvasculature.</p>

<p><strong>Purpura:</strong>&nbsp;spots of variable size on the skin or in the mucous membranes, due to small hemorrhages; they can be dark red and as small as a pinhead (petechiae) or purplish and 2 to 3 cm in diameter (ecchymosis).</p>

<h2><span style="color:#6f1813;">Types of TTP</span></h2>

<h2><span style="color:#6f1813;">Primary aTTP vs secondary aTTP<sup>2,6,7</sup></span></h2>

<table border="1" cellpadding="1" cellspacing="1" style="width:100%;">
 <tbody>
  <tr>
   <td style="background-color: rgb(241, 135, 0);">
    <p style="text-align: center;"><span style="color:#ffffff;"><strong>TTP causes systemic microthrombi and consequent thrombocytopenia, hemolytic anemia, and organ ischemia. These symptoms—coupled with TTP’s rarity—can cause confusion with other TMAs, making TTP a challenge to diagnose.<sup>8-10</sup></strong></span></p></td>
  </tr>
 </tbody>
</table>

<h2><span style="color:#6f1813;">Signs and symptoms of aTTP include<sup>1,7-10</sup></span></h2>

<p>Petechiae, purpura, bruising (skin)</p>

<p>Headache, confusion, seizures, coma<br>
  (neurological)</p>

<p>Abdominal pain, diarrhea<br>
  (gastrointestinal)</p>

<p style="text-align: center;"><strong>Suspect aTTP?</strong></p>

<h2 style="text-align: center;"><span style="color:#6f1813;">The incidence of TTP is 2 to 6 per million individuals<sup>4</sup></span></h2>

<h2 style="text-align: center;"><span style="color:#6f1813;">aTTP primarily affects young, healthy adults<sup>13,14</sup></span></h2>

<p><strong>years of age on average</strong></p>

<p><strong>of people affected are women</strong></p>

<p><strong>higher aTTP incidence<br>
   in the Black population</strong></p>

<h2 style="text-align: center;"><span style="color:#6f1813;">If left untreated, TTP is rapidly fatal<sup>8,15-17</sup></span></h2>

<p><strong>acute mortality rate<sup>16,17</sup></strong></p>

<p><strong>of TTP deaths occur within 24 hours of presentation<sup>8</sup></strong></p>

<h3 style="text-align: center;"><span style="color:#6f1813;">Even with plasma exchange therapy (PEX), mortality risk persists<sup>15,16,18</sup></span></h3>

<h2><span style="color:#6f1813;">The burden of thrombotic thrombocytopenic purpura (TTP)</span></h2>
<p>Short- and long-term implications threaten patients with aTTP</p>
<h3><span style="color:#6f1813;">Short-term implications</span></h3>

<p style="text-align: center;"><strong>An increased time at risk of microvascular thrombosis can lead to irreversible organ damage to the heart, brain, and kidneys<sup>16</sup></strong></p>

<h3><span style="color:#6f1813;">Long-term implications</span></h3>

<table border="1" cellpadding="1" cellspacing="1" style="width:100%;">
 <tbody>
  <tr>
   <td style="background-color: rgb(241, 135, 0); text-align: center;"><span style="color:#ffffff;"><strong>Long-term morbidity leads to suboptimal health-related quality of life, in both mental (anxiety, depression) and physical functioning, and potentially reduces life expectancy<sup>8-20</sup></strong></span></td>
  </tr>
 </tbody>
</table>

Abbreviations

<h2><span style="color:#6f1813;">The pathophysiology of thrombotic thrombocytopenic purpura (TTP)</span></h2>
<h3><span style="color:#6f1813;">TTP is characterized by potentially fatal systemic microthrombi<sup>1,2</sup></span></h3>
<p>TTP is caused by severely decreased activity of the ADAMTS13&nbsp;enzyme. ADAMTS13 is the protease that cleaves von Willebrand factor (vWF). In patients with acquired TTP (aTTP), this deficiency is immune mediated.<sup>1</sup></p>
<p>Decreased ADAMTS13 activity leads to an accumulation of ultra-large von Willebrand factor (ULvWF) multimers, which spontaneously bind to platelets and induce aggregation. This results in dangerous microthrombi that drive severe thrombocytopenia, microangiopathic hemolytic anemia, and organ ischemia.<sup>2</sup></p>
<p>ADAMTS13=a disintegrin and metalloproteinase with thrombospondin type 1 motif, 13.</p>
<h4><span style="color:#6f1813;">TTP pathophysiology</span></h4>
<p>An in-depth exploration of the causes of TTP and the severe consequences of the disease.</p>

<p>TTP is a life-threatening thrombotic microangiopathy (TMA) that requires quick diagnosis and urgent treatment.&nbsp;<sup>1,2</sup></p>

Understanding TTP

<h2><span style="color:#6f1813;">What is the pathophysiology of aTTP?</span></h2>
<h3><span style="color:#6f1813;">3 Key mechanisms drive the pathophysiology of aTTP<sup>2</sup></span></h3>

<p><strong>Auto antibody formation</strong><br>
  Autoantibodies to ADAMTS13 inhibit its activity or enhance clearance of the enzyme.</p>

<p><strong>ADAMTS13 deficiency causes&nbsp;uncleaved ULvWF</strong><br>
  ADAMTS13 (the enzyme responsible for cleaving vWF in the blood-clotting process) is deficient, leaving platelet-hyperadhesive ULvWF strands intact to circulate without being cleaved.</p>

<p><strong>Microthrombi formation</strong><br>
  ULvWF accumulates and spontaneously binds to platelets, forming microthrombi.</p>

<h3><span style="color:#6f1813;">Normal Physiology<sup>2,3</sup></span></h3>

<h3><span style="color:#6f1813;">Thrombotic Thrombocytopenic Purpura<sup>2,3</sup></span></h3>

<p data-font="legal">Adapted from Joly B et al.&nbsp;Blood&nbsp;2017 and Saad J &amp; Schoenberger L 2018.</p>

<h2><span style="color:#6f1813;">Formation of microthrombi causes<sup>2</sup></span></h2>

<h4>Severe thrombocytopenia</h4>
<p>(often platelets &lt;30 × 109/L)</p>

<h4>Microangiopathic hemolytic anemia<br>
  (MAHA)</h4>
<p>characterized by the presence of schistocytes in blood smear</p>

<h4>Organ ischemia</h4>
<p>(lesions can occur in any organ, but they most frequently affect the heart, brain, gastrointestinal tract, and kidney)</p>

<table border="1" cellpadding="1" cellspacing="1" style="width:100%;">
 <tbody>
  <tr>
   <td style="text-align: center; background-color: rgb(241, 135, 0);"><span style="color:#ffffff;"><strong>Platelet aggregation leads to microthrombi formation, which can have fatal consequences.<sup>1,2</sup></strong></span></td>
  </tr>
 </tbody>
</table>

<h2><span style="color:#6f1813;">Signs and symptoms of aTTP</span></h2>
<p><strong>The symptoms of aTTP are driven by microthrombi, which are unpredictable and potentially fatal<sup>1,2</sup></strong></p>
<p>Microthrombi can have serious acute and chronic consequences in TTP—putting patients at risk for devastating effects and death without urgent treatment.</p>

<h3 style="text-align: center;"><span style="color:#6f1813;">Microthrombi can occur in any organ with microvessels, but the most commonly affected are<sup>3</sup></span></h3>

<p data-font="legal">Tissue and organ damage resulting from ischemia leads to increased levels of lactate dehydrogenase (LDH), troponins (heart), and creatinine (kidney).¹,⁴</p>

<h2><span style="color:#6f1813;">Expert Opinion About aTTP</span></h2>

<h2><span style="color:#6f1813;">Signs and symptoms of acquired thrombotic thrombocytopenic purpura (aTTP) include<sup>1,4,5</sup></span></h2>

<p><strong>of TTP episodes result in ischemia due to platelet aggregation in the microcirculation of the brain.<sup>3</sup></strong></p>

<h2><span style="color:#6f1813;">The consequences of microthrombi can be severe, with TTP potentially resulting in acute thromboembolic events such as<sup>1,2,4</sup></span></h2>

<h4>&nbsp;</h4>
<h4>Arterial thrombosis</h4>

<h2><span style="color:#6f1813;">Consequences can also be long term</span></h2>

TTP is a life-threatening thrombotic microangiopathy (TMA) that requires quick diagnosis and urgent treatment. <sup>1,2</sup>

ACQUIRED-THROMBOTIC-THROMBOCYTOPENIA-PURPURA-ATTP

RARE-BLOOD-DISORDERS

You can imagine more than 90% of CRSwNP patients losses their ability's of smell

<p>Smell is mediated by Olfactory Sensory Neurons in the olfactory epithelium. Horizontal Basal Stem Cells regenerate these Olfactory Sensory Neurons, maintaining the sense of smell.</p>

CHRONIC-RHINOSINUSITIS-WITH-NASAL-POLYPS-CRSWNP

RESPIRATORY

“Smell—we call it the Cinderella of the senses"

<p>In dealing with the severe burden of CRS with nasal polyps, patients can get used to their reduced sense of smell. What would it mean to bring it back? Watch as an expert explains the impact the sense of smell has on quality of life and what biologics can do to restore patients’ lost smell.</p>
<p>Restoring the sense of smell is key to improving quality of life for patients with CRS with nasal polyps. For patients experiencing significant smell loss, biologic therapy can help restore the sense of smell.<sup>1,2</sup></p>

Scientific Content

Latest Articles

Understanding TTP

You can imagine more than 90% of CRSwNP patients losses their ability's of smell

“Smell—we call it the Cinderella of the senses"