Have you ever heard of acquired thrombotic thrombocytopenic purpura? José Pedro Carda, a hematologist at Hospital da Luz, explains everything you need to know.
Every year, on October 13th, World Thrombosis Day is celebrated. José Pedro Carda, a hematologist at Hospital da Luz, explains that the date was "created with the aim of drawing attention to the risks and ways of preventing this disease".
In case you don't know, "thrombosis describes the formation of clots inside veins or arteries, which prevents the total or partial circulation of blood, with complications depending mainly on the organ affected".
According to the doctor, "one of the rarest causes of thrombosis - acquired thrombotic thrombocytopenic purpura (aTPT) - was described 100 years ago by Eli Moschcowitz".
"It took decades of research to understand the pathophysiological bases of a disease that was once fatal, but now with approaches that have radically improved the prognosis", he adds. It is "a very rare but potentially fatal disease that affects blood clotting."
© José Carda
It is characterized by the "presence of small clots in blood vessels, leading to a decrease in platelets, severe anemia and multiple symptoms such as fatigue, paleness, small purple spots on the skin, abdominal pain and neurological changes, such as confusion or headache". It is these last symptoms that usually take the patient to the emergency department, "with symptoms suggestive of, but simulating, a stroke".
This means that "early recognition of these symptoms is crucial" because "rapid diagnosis and treatment are crucial to the patient's recovery".
Generally, "medical investigation will include blood tests, which are essential to confirm the suspicion of aPTT, and if confirmed, referral to a specialist and immediate initiation of treatment are essential".
According to the specialist, "time is a critical factor: delays in diagnosis can lead to serious complications, including organ damage and, in extreme cases, death, due to complications of thrombotic phenomena in the kidneys, heart or brain".
Unfortunately, “patients who experience an episode of aPTT can face a significant impact on their lives in the long term,” she explains. “Although many make a full recovery, some patients may experience after-effects, such as neurological problems or chronic fatigue.”
In addition, “the experience of dealing with a serious, potentially fatal illness can generate anxiety and emotional disturbances, affecting quality of life.” With this in mind, “psychological support and rehabilitation are important aspects of recovery, helping patients to reintegrate their daily routines.”
In “recent years, there have been significant innovations in the diagnosis and treatment of aPTT,” says the doctor. For example, “early interventions are currently dependent on faster and more accurate methods for identifying the disease, techniques that are available in several national institutions.”
She also explains that “early diagnosis allows for early treatment” and “therapeutic approaches in this field have evolved, providing options that minimize risks and significantly improve outcomes for patients.”
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