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What is Thrombotic Thrombocytopenic Purpura?
Thrombotic thrombocytopenic purpura (TTP) is a clinical syndrome defined by the presence of thrombocytopenia and microangiopathic hemolytic anemia. There are many possible causes, including E. coli O157:H7, all of which act through the common mechanism of inducing endothelial cell damage.
History of the Clinical Recognition of TTP
TTP was first recognized by clinical features in 1925, but it was not until 1966 that Amorosi and Ultmann described the diagnostic pentad of symptoms that classify TTP today.
Classification of TTP
Patients are considered to have “primary” Thrombotic Thrombocytopenic Purpura if they have no clinically apparent illness that is recognized to be associated with this syndrome. In an attempt to identify the other subsets of patients with TTP with similar characteristics, several classifications have been proposed.

Symptoms and Risks of Thrombotic Thrombocytopenic Purpura

What are the typical symptoms of Thrombotic Thrombocytopenic Purpura?
The presenting symptoms of TTP are markedly variable. Regardless of the main symptoms at presentation, laboratory studies invariably indicate anemia and a variable degree of renal involvement in patients with Thrombotic Thrombocytopenic Purpura.
Renal Involvement in Patients
A high percentage of patients classified as having Thrombotic Thrombocytopenic Purpura have renal (kidney) failure.
CNS (Neurologic) Involvement
The nature of the neurologic signs and symptoms can include varying degrees of mental syndromes, from confusion to stupor and coma, hemi sensory findings and even seizures. Changes in mental function are noted to be the most frequent initial neurologic manifestation of Thrombotic Thrombocytopenic Purpura, but are less clinically apparent in children than in adults.
Cardiac Involvement
Since TTP is a diffuse disease involving virtually every organ in the body, and because some of the patients studied had cardiac rhythm disturbances, as well as sudden cardiac arrest, it was important to investigate the potential effects of this disease on the heart and the conduction system.

Treatment of Thrombotic Thrombocytopenic Purpura

How is Thrombotic Thrombocytopenic Purpura treated?
Today, the treatment of Thrombotic Thrombocytopenic Purpura involves a daily single plasma volume exchange (plasmapheresis), which is continued until the platelet count normalizes and there is minimal hemolysis. Seriously ill patients may receive fresh frozen plasma infusions (without exchange) for several hours until plasmapheresis can be arranged.
Treatment Outcomes in Patients with TTP
With respect to both treatment and prognosis of TTP, there is a need to determine whether early intervention with any treatment can improve the rate of initial recovery, decrease the frequency of exacerbations and relapses, and decrease the risk for chronic renal failure. Long-term clinical follow-up studies on the outcomes of patients after recovery from an acute episode of Thrombotic Thrombocytopenic Purpura are necessary to document the risk for relapse, as well as the occurrence of renal failure and hypertension.

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