Thrombotic thrombocytopenic purpura
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Clinic
- TTP is a blood disorder that results in blood clots forming in small blood vessels throughout the body.
- This results in a low platelet count, low red blood cells due to their breakdown, and often kidney, heart, and brain dysfunction.
- Symptoms may include large bruises, fever, weakness, shortness of breath, confusion, and headache.
- Known triggers include Bacterial infections, Certain medications, Autoimmune diseases such as lupus, and Pregnancy.
- The underlying mechanism typically involves antibodies inhibiting the enzyme ADAMTS13
- Less commonly TTP is inherited
Signs / Symptoms
- Influenza-like or diarrheal illness before developing TTP.
- Neurological symptoms are very common and vary greatly in severity. Feeling very tired, Confusion, Headaches. Seizures and symptoms similar to those of a stroke can also be seen.
- Other symptoms include Jaundice or paleness of the skin, Fast heart rate or Shortness of breath, Petechiae.
- Fever
- Changes in mental status
- Thrombocytopenia
- Reduced kidney function
- Hemolytic anemia
- Hypertension
Causes
Autoimmune
- Not to be confused with Immune thrombocytopenic purpura.
- TTP of unknown cause was long known as idiopathic TTP but in 1998 the majority of cases were shown to be caused by the inhibition of the enzyme ADAMTS13 by antibodies. The relationship of reduced ADAMTS13 to the pathogenesis of TTP is known as the Furlan-Tsai hypothesis, after the two independent groups of researchers who published their research in the same issue of the New England Journal of Medicine. These cases are now classed as an autoimmune disease and are known as autoimmune TTP (not to be confused with immune/idiopathic thrombocytopenic purpura).[citation needed]
Genetic
Autosomal recessive manner.
Secondary
- Cancer
- Bone marrow transplantation
- Pregnancy
- Medications
- HIV-1 infection
Remedies
- ↑ Dhingra G, Maji M, Mandal S, Vaniyath S, Negi G, Nath UK. COVID 19 infection associated with thrombotic thrombocytopenic purpura. J Thromb Thrombolysis. 2021 Aug;52(2):504-507. doi: 10.1007/s11239-021-02452-0. Epub 2021 Apr 27. PMID: 33904053; PMCID: PMC8075832.