What causes acquired thrombotic thrombocytopenic purpura?
Acquired TTP is caused when a person’s body mistakingly makes antibodies that block the activity of the ADAMTS13 enzyme. THe ADAMTS13 enzyme normally helps control the activity of certain blood clotting factors.
How can you tell the difference between HUS and TTP?
HUS is characterized by thrombocytopenia, anaemia and renal insufficiency, whereas the pentad of signs and symptoms including thrombocytopenia, anaemia, neurologic deficit, renal dysfunction and fever is observed in TTP.
Which disease is associated with TTP?
TTP can also cause red blood cells to break apart faster than your body can replace them. This leads to a rare form of anemia called hemolytic anemia. TTP can be fatal. Without treatment, it can cause long-term problems, such as brain damage or a stroke.
What are the laboratory findings seen in TTP?
TTP patients usually present with a characteristic set of laboratory findings. This includes a negative direct antiglobulin test (DAT), microangiopathic hemolytic anemia (MAHA), and thrombocytopenia, which are considered major laboratory criterion of TTP.
What is thrombotic thrombocytopenia purpura?
Description. Collapse Section. Thrombotic thrombocytopenic purpura is a rare disorder that causes blood clots (thrombi) to form in small blood vessels throughout the body. These clots can cause serious medical problems if they block vessels and restrict blood flow to organs such as the brain, kidneys, and heart.
What is the difference between TTP and DIC?
Thrombotic thrombocytopenic purpura (TTP) – hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy superficially like DIC, but distinctly different; in contrast to DIC, the mechanism of thrombosis is not via the tissue factor (TF)/factor VIIa pathway. Results of blood coagulation assays in TTP-HUS are normal.
What causes ADAMTS13 deficiency?
ADAMTS13 deficiency can be acquired or congenital Secondary (23 to 67 percent of cases), arising from a variety of conditions, including autoimmune disorders, solid organ or hematopoietic cell transplant, malignancy, drugs and pregnancy.
Is Coombs test positive in TTP?
In TTP, hemolytic anemia occurs because red blood cells are broken into pieces as they try to squeeze around blood clots. When TTP is the cause of hemolytic anemia, the Coombs test is negative. The test is positive if antibodies (proteins) are destroying your red blood cells.
What level of TTP is ADAMTS13?
An ADAMTS13 activity level of less than 10% supports the diagnosis of TTP in appropriate clinical contexts, but many centers do not offer testing in-house and must send out the test to a reference laboratory with a turnaround time of several days.
What infections cause low platelets?
Infections with protozoa, bacteria and viruses can cause thrombocytopenia with or without disseminated intravascular coagulation. Commonly dengue, malaria, scrub typhus and other rickettsial infections, meningococci, leptospira and certain viral infections present as fever with thrombocytopenia.
Is D-dimer elevated in TTP?
D-dimers are indicative of fibrinolysis and thus, thrombin activation, which usually is normal or mildly elevated in patients with TTP.
Does TTP cause low fibrinogen?
We observed a slight increase of fibrin-fibrinogen degradation products (FDP) in the plasma of four patients during the acute stage of TTP, but the level of the D-dimer remained within normal variation and was extremely low compared with that in 27 samples from patients with DIC showing the same level of FDP.