Clinical study on 20 cases of acquired thrombotic thrombocytopenic purpura caused by different inducements / 重庆医学

ABSTRACT

Objective To enhance the recognition of thrombotic thrombocytopenic purpura(TTP) by analyzing the clinical data of acquired TTP .Methods The clinical features ,laboratory detection ,treatment strategy and outcome of 20 patients with ac‐quired TTP were retrospectively analyzed .Results Among 20 patients with acquired TTP ,16 cases had the inducing factors ,inclu‐ding abnormal autoimmune ,systemic lupus erythematosus(SLE) ,antiphospholipid antibody syndrome(APS) ,viral infection ,bacte‐rial infection ,medication ,lymphoma and allogenic hematopoietic stem cell transplantation .When the patients were diagnosed firstly , only 5 cases(25% ) had typical pentalogy of TTP ,18 cases(90% ) were complicated with thrombocytopenia ,14 cases(70% ) were complicated with microangiopathic hemolytic anemia ,17 cases (85% ) with neurologic abnormalities ,15 cases (75% ) fever and 8 ca‐ses(40% ) with renal insufficiency .Sixteen cases(80% ) had high percentage of reticulocytes ,the schistocytes were detected in the peripheral blood smears of 18 cases ,among them microspherocytes were simultaneously detected in 3 cases .Twenty cases had high level of lactate dehydrogenase .All the cases received the therapy of plasmatherapy (plasma exchange and/or plasma infusion) as the principal thing ,among them 9 cases received the glucocorticoid therapy ,2 cases received the cytoxan therapy ,2 cases received the human immune globulin therapy and 4 cases received the low‐dose rituximab therapy (100 mg ,once per week ,for consecutive 4 times) .Eleven cases (55% ) reached complete remission(CR) ,3(15% ) cases got partial remission(PR) and 4(15% ) cases died . The total effective rate was 70% .Three cases(21% ) out of 14 effective cases relapsed .Conclusion The diagnosis of TTP is based on the comprehensive analysis of clinical data .Early diagnosis and early treatment of plasmatherapy as the main means can improve the prognosis of patients with TTP .