عنوان مقاله [English]
The thrombotic thrombocytopenic purpura (TTP) is a rare and classic disorder of hematopoietic tissue with a high mortality rate if untreated or delayed therapy, whether immediate diagnosis and promptly treated with plasmapheresis can change the grave prognosis.
Materials and Methodes
This is aretrospective analysis that was performed on clinical characteristics and treatment outcome of 21 patients diagnosed and treated with plasmapheresis, in shahid sadoughi hospital.
The most common presenting symptom was fatigue, sickness followed by fewer and purpura, lab result showed that all patients had increased LDH , thrombocytopenia , 95% had anemia (with decreased HB &HCT), 90% of patient had increased retic(reticolocytosis), 67% had increased indirect bilirobin level, 62% had neurological disorders, renal function abnormality in 71%. All Patients were treated with a daily plasma exchange, 90 of these patients achieved remission that 76% had complete response, 14% had partial response and 5% had no response because of their complicated underline diseases. 76% of patient had complete response at platelet, 19/2% had partial remission of thrombocytopenia and about 90% of patient had complete remission of renal dysfunction.
However TTP is a disorder with a high mortality but it is described that plasmapheresis can treat TTP in most patients affectively; even in patients who don’t achieved complete remission, prolonged plasmapheresis with adjutive therapy could improve them. So based on this study we suggest that plasmapheresis should be done immediately after diagnosis of TTP or if no remission achieved we should think about underline diseases.