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Therapeutic plasma-exchange (TPE) is used as primary and adjunctive therapy in treatment of several hematologic diseases. We retrospectively evaluated the results of TPE in hematologic diseases during 2008-2012. A total of 301 TPE procedures were performed in 44 patients (19 male and 25 female, with mean age of 50.6±15years). Fifteen of 44 patients had thrombotic thrombocytopenic purpura (TTP), 14 patients had HELLP syndrome (Hemolysis Elevated Liver enzymes, Low Platelet count), 10 patients had multiple myeloma-hyperviscosity and the rest five patients had snake bite. Fresh frozen plasma (FFP) was used as replacement fluid. Complete response (CR) was achieved on 13 patients (87%) in primarily TTP. CR was achieved in all other three diseases. Total complications were detected in 8.1% of the TPE procedures. Adverse events (AEs), were seen in 5.4% of all procedures. None of the patients died from any complication. AE occurred in 4% (Grade-I), 1% (Grade-II), and 0.3% (Grade-III) of the procedures. The most common AE were nausea/vomiting, hypotension, pruritus and abdominal pain. TPE is effectively and safely carried out in our center in hematologic diseases., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

Thrombotic thrombocytopenic purpura (TTP) is a classic, but not a common disorder of hematology. Plasma exchange (PE) was shown to nearly reverse its 90% mortality rate. However, there are still some fatal outcomes in this dramatic disease. We present our experience of plasma exchange plus corticosteroids for the treatment of TTP in our hospital. Patients with TTP diagnosed between January 1996 and January 2005 were identified by a retrospective review of records of the Uludag University Hospital, Bursa (the largest referral center for adults with this disorder in this region with an estimated 2.2 million residents), which performs all therapeutic PE in the southern Marmara region in Turkey. A total of 11 (6 male, 5 female) patients were treated for TTP during this period. The median age was 39 years (range 18-49). One plasma volume exchange daily plus steroid was the principle treatment in all patients. A total of 295 PE sessions were performed. We have obtained six complete responses (CR) and three partial responses (PR) with daily PE and steroid (response rate 9/11). One of our primary refractory patients was saved with pulse steroid+cyclosporine+vincristine. Now, he is disease free for over one year. The other refractory patient did not develop any response to salvage therapy and expired on day 15 with status epilepticus and ventilator related pneumonia (mortality rate 1/11). A CR was obtained with adjuvant treatments in all three PR patients. Only one CR patient developed an early relapse (early relapse rate in CR patients 1/6). She was treated successfully with daily PE plus vincristine. Our median follow up period was 25 months (range 9-108). Considering our local population, our annual incidence is only about 0.63 new cases per one million people. This figure is considerably less than the data from US, which indicated an incidence of 3.7 cases per 1,000,000. To our knowledge, there is no high variability in the incidence of TTP in the different geographical regions of the world. It suggests that considerable number of patients escaped notice. We hope that, demonstrating the successful outcome, this article serves to urge primary physicians to keep in mind the diagnosis of TTP and refer suspected cases quickly.

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.

In this retrospective, nonrandomized study, we describe our experience in the management of 29 consecutive patients with thrombotic thrombocytopenic purpura (TTP) treated with a combined therapy of plasma exchange (PE) and steroids at a single center. We compared the effectiveness of high-dose steroids (20-25 mg/kg methyl prednisolone) as first-line treatment in combination with PE therapy with the combination of standard-dose steroids (1 mg/kg methyl prednisolone) and PE in adult patients with TTP. Clinical, laboratory data and treatment outcomes such as response rate, median time to recovery and survival were evaluated retrospectively. Overall (OR) and complete (CR) response rates were 69 and 52% respectively. Similar response rates were found in patients treated with pulse or conventional dose steroids; however, the median time to response was delayed in the high-dose methyl prednisolone (HDP) group. The median time from the initiation of symptoms to initiation of treatment was approximately 15 days (range: 0-30). Delayed treatment in our patients because of delayed referral to our center resulted in poor response to treatment. In all, four of 14 (27%) complete responders experienced relapses. The predicted relapse rate was 48% at a median of 30 months. All the relapses presented with a combination of thrombocytopenia and microangiopathic hemolytic anemia. This analysis showed that high-dose steroid treatment did not prove to be beneficial for TTP patients as firstline therapy combined with PE. Moreover, pulse steroid interventions might have resulted in delayed responses and our data suggest that initiation of treatment with PE should not be delayed.