Local experience with thrombotic thrombocytopenic purpura from the western part of Turkey.

摘要

Thrombotic thrombocytopenic purpura (TTP) is a fatal disorder if left untreated. Therapeutic plasma exchange (PE) has resulted in excellent remission and survival rates in these patients but there is a need for alternative immuno-modulatory treatments in unresponsive patients. We present a descriptive, retrospective study of 25 (14 female, 11 male) adult patients admitted to our hematology unit with TTP. The patients' median age was 32 years. The patients were treated with immediate PE and a standard dose of corticosteroid therapy. Twenty percent of the patients obtained a complete response after the addition of Intravenous immuno-globulin (IVIg). Improvement of clinical status, platelet counts and serum LDH levels were seen after a median 13, 14 and 3.5 PE sessions, respectively. The mortality rate was 12%. One patient has a chronic relapsing form of TTP and two patients have relapsed. All the other patients are still in complete remission after a median of 7 years of follow-up. Although PE therapy is life-saving and the application of early corticosteroid treatment could obtain early and durable responses, addition of other immuno-modulatory treatments are needed in unresponsive patients. The use of IVIg could result in a dramatic response.