گزارش 28 مورد بیمار مبتلا به ترومبوتیک ترومبوسیتوپنیک پورپورا (TTP) و بررسی عوامل پیش آگهی و خطر در درمان بیماری

Thrombotic thrombocytopenic purpura is the result of multiple thrombus disseminated in small arterioles throughout the body. Principle damage is in the endothelium of small vessels and capillaries. From 1982 to 1999, 28 patients (16 female and 12 male) have been treated for TTP. The outcome was evaluated for risk factors and prognosis. These patients were 18 to 69 years old. All had neurological sings, fever, microangiopathic hemolytic anemia and thrombocytopenia. These patients did not have predisposing factors. All patients were negative for hepatitis C and B. Plasmaphoresis was the main treatment in all and was done. The patients had plasmaphoresis from 1.5 to 6 liters per day. Minimum requirement was 4.5 liters to 95 liters in all. Replacement was fresh frozen plasma in 16 patients. 12 patient had FFP and cryopoor plasma half-and-half for replacement. 22 patients completed the treatment and became well 6 patients died. Chronic head ache and progressive dementia was observed in 2 patients. 6 patients did not recover and died. Platelets count over 100000/mm LDH lower than 500 units and disappearance of neurological disturbance was the end point of treatment. This study showed the good result of plasmaphoresis in TTP ad graves outcome in patient with cerebral bleeding, cardiopulmonary failure, prolonged QT in EKG, hypotension, and increased CPK. We did not observe any chronic cases in these groups. The cause of death were cerebral hemorrhage in 2 respiratory failure in 3 patients. One of the these patients had adult respiratory distress syndrome and pneumonia in another patient.