T.T.P. [Moschcowitz disease]
Thrombotic thrombocytopenic purpura (TTP or Moschcowitz disease) is a rare disorder of the blood-coagulation system, causing multiple blood clots to form in blood vessels around the body.
Most cases of TTP arise from deficiency or inhibition of the enzyme ADAMTS13, which is responsible for cleaving large multimers of von Willebrand factor. This leads to hemolysis and end-organ damage, and may require plasmapheresis therapy.

TTP occurs as the result of the lack of an enzyme in the plasma portion of the blood that is responsible for cleaving, or cutting up, a large molecule called Von Willebrand factor (vWF) that is involved in blood clot formation. In true TTP, a lack of this enzyme allows ultra large vWF molecules to circulate in the blood and leads to the inappropriate formation of platelet clumps (thrombi) particularly within blood vessels supplying the brain and kidneys. These give rise to the typical symptoms of TTP. HUS, on the other hand, also involves the formation of micro-thrombi, but through a process that does not involve abnormal vWF activity. The treatment for true TTP involves plasma exchange, which is done in an effort to replace the enzyme that cleaves vWF. In HUS, plasma exchange is unnecessary, but dialysis is often done when the kidneys fail to filter normally due to the presence of micro-thrombi within the tiny blood vessels at the heart of the kidneys filtering units.


ABOUT-TTP.COM

WIKI
Valid XHTML 1.0 TransitionalValid CSS!