{ event: "article_read", name: `Signs and symptoms of aTTP`, author: ``, tags: `Sällsynt blodsjukdom | Acquired thrombotic thrombocytopenic purpura`, publication_date: ``, interaction_type: "content" } 
Signs and symptoms of aTTP
Know the signs and symptoms of acquired TTP (aTTP).
In patients with acquired TTP (aTTP), immune-mediated deficiency of ADAMTS13 results in dangerous microthrombi that cause:1-3
Severe thrombocytopenia
(platelets < 30x109/L)
Microangiopathic hemolytic anemia (MAHA)
Characterized by the presence of schistocytes
Tissue ischemia
Lesions can occur in any organ, but most frequently affect the heart, brain and kidneys
The consequences of microthrombi can be serious, and aTTP may lead to acute thromboembolic events such as:1,4,7
Myocardial infarction
Stroke
Arterial thrombosis
Early death
Signs and symptoms of aTTP1,2,4-6
These symptoms are only a limited part of what can be observed during an TMA/ aTTP episode. The aspecific symptoms and the variety in phenotype must be taken into account.
Adapted from Scully, M. et al.4
Skin
Petechiae, purpura, bruising
Neurological
Headache, confusion, seizures, coma
Cardiac
EKG abnormalities
Gastrointestinal
Abdominal pain, diarrhea
Kidney
Proteinuria/hematuria
Dr. Scully details the signs and symptoms of the disease
References
- Joly BS, et al. Thrombotic thrombocytopenic purpura. Blood. 2017;129(21):2836–46.
- Tsai H-M. Pathophysiology of thrombotic thrombocytopenic purpura. Int J Hematol. 2010;91(1):1-19
- Sadler JE. Pathophysiology of thrombotic thrombocytopenic purpura. Blood. 2017;130:1181–8.
- Scully M, Hunt BJ, Benjamin S, et al; British Committee for Standards in Haematology. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol. 2012;158(3):323-335
- Azoulay E, Bauer PR, Mariotte E, et al; Nine-i Investigators. Expert statement on the ICU management of patients with thrombotic thrombocytopenic purpura. Intensive Care Med. 2019;45(11):1518-1539
- Gallan AJ, Chang A. A new paradigm for renal thrombotic microangiopathy. Semin Diagn Pathol. 2020;37(3):121-126
- Goel R, et al. Prognostic risk-stratified score for predicting mortality in hospitalized patients with thrombotic thrombocytopenic purpura: nationally representative data from 2007 to 2012. Transfusion. 2016;56(6):1451-1458.
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