Thrombotic Thrombocytopenic Purpura
Thrombotic Thrombocytopenic Purpura (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. Repeated episodes may occur.
It is clinically and pathologically similar to hemolytic Uremic Syndrome (HUS), with little variation. This is also a thrombotic micro angiopathy.
Diagnosis of Thrombotic Thrombocytopenic Purpura
(1) Presence of fever (frequent)
(2) Age (young adults)
(3) Neurologic abnormalities tend to predominate (e.g. stroke) and cause death.
(4) Lesser degree of renal involvement compared to HUS (ARF) occurs in about 10% case only), Presence of hematuria (common), Proteinuria < 5gm / day, Serum creatinine more than 2 mg / dl in only 50% cases
(5) Histology : shows similar renal lesion like HUS.
Treatment of Thrombotic Thrombocytopenic Purpura
I.V. infusion of prostacyclin
Plasma exchanges vigorously
Combination of plasma exchange, fresh plasma antiplatelet drugs and steroids have shown shown dramatic response in some cases.
Splenectomy in refractory cases.
Prognosis of Thrombotic Thrombocytopenic Purpura
75% mortality within 3 months
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