Rituximab may be used to treat patients with thrombotic thrombocytopenic purpura (TTP) refractory to plasma exchange or recurrent disease. While initial response rates are reported to be high, long-term follow-up data of patients treated with rituximab are not available to date however important to estimate the safety and benefit of this treatment. 12 patients with non-familial idiopathic TTP refractory to plasma exchange or with recurrent disease treated with rituximab between 2000 and 2008 were re-examined. The median follow-up was 49.6 months, ranging from 11 to 97 months. All patients achieved initial complete remission after application of rituximab. During follow-up, nine patients remained disease-free and three patients suffered from recurrent disease. All patients with recurrent disease responded to subsequent rituximab therapy. No long-term side effects were noted during the follow-up period. In conclusion, rituximab represents an effective second-line treatment option in relapsing or refractory TTP. Still, patients need to be closely monitored for relapses with extended follow-up.Response to Reviewers: Reviewer #11. Abstract, line 2: I think "acceptable" is not appropriate until larger studies are done. I would suggest changing to: "Rituximab may be used to treat relapsed or refractory TTP" Changes were made accordingly. 2.Abstract, line 5: While initial response rates are "reported to be" high..Changes were made accordingly.3. Abstract, line 32: "Still, patients need to be closely monitored as the relapse rate increases with longer follow-up." Change to ".monitored for relapses with extended follow-up".Changes were made accordingly. 4.Introduction, line 41: Remove "not otherwise explained"Changes were made accordingly. 5.Introduction, line 51: Revise: "Acquired idiopathic TTP is differentiated from hereditary TTP (Upshaw-Schulman syndrome)." ie how are they differentiated?The differentiation between acquired and congenital TTP follows in detail in the next paragraph: "Large progress was made in recent years in understanding the pathophysiology of TTP. Moake et al. described unusually large von Willebrand factor (VWF) multimers in the plasma of patients with relapsing acquired or congenital TTP causing intravascular platelet aggregation occluding the microvasculature [2]. Tsai et al. [3] and Furlan et al. [4] independently identified a VWF-cleaving protease (ADAMTS13) missing from the plasma of patients with congenital TTP [5] and severely deficient in patients with acquired idiopathic TTP. Mutations in the ADAMTS13 gene were shown to cause congenital TTP [6], whereas IgG autoantibodies inhibit the enzyme in most cases of the acquired form of the disease [7][8]." To avoid repetitions we left this part of the introduction as it was. If the editor has a distinct opinion to this point, we are more than willing to change it. 6.Introduction, line 12 (2nd page of introduction) and throughout the paper: "plasma exchange against fresh frozen plasma" change to ". WITH fresh frozen plasma."Changes w...
Background:Inframammary fold reconstruction has scarcely been evaluated in literature. No biomechanical analyses have been performed comparing different reconstructive methods. This evaluation compares the gold-standard suture reconstruction with an intrarib anchor system (Micro BioComposite SutureTak, Arthrex).Methods:Three analysis groups were compared including 8 Sawbone blocks, 22 embalmed cadaver, and 27 regular cadaver specimens (N = 57). Transient mechanical analysis was performed at 5 N/s using an Instron 5565 test frame.Results:Ultimate load favored the anchor system (compared with the gold-standard suture) by a factor of 9.8 (P < 0.0001) for the regular cadaver group and a factor of 1.7 (P < 0.038) for the embalmed cadaver group. A similar statistically significant benefit was shown for stiffness and load at 2-mm displacement.Conclusions:This analysis showed an anchor system to be the biomechanically superior fixation method in terms of ultimate load, fixation stiffness, and displacement at failure when compared with the gold-standard suture method in inframammary fold reconstruction. Because of superior stability in every aspect, an anchor system may be considered for inframammary fold reconstruction.
3513 Poster Board III-450 Introduction Rituximab is an accepted treatment modality for patients with TTP refractory to plasma exchange or recurrent disease. While initial treatment response rates are at a high level, long-term follow-up of patients treated with Rituximab is not accessible to date. However this data implies important information, since overall and progression free survival, side effects and a possible need for a maintenance therapy must be taken in consideration. Methods 12 patients with TTP refractory to plasma exchange or with recurrent disease treated with Rituximab at the haematology department of the University of Cologne between 2000 and 2008 were re-examined. All patients suffered from non-familial idiopathic TTP, cases with secondary TTP following stem cell transplantation or underlying diseases such as cancer or rheumatologic disorders were excluded. Additional to physical examination, ADAMTS13 activity and the presence of an ADAMTS13 inhibitor were analyzed. Results The median age of the patients was 43.2 years and 75% of the patients were female. The majority of patients received Rituximab either because of refractory disease or severe allergic reactions during standard therapy with plasma exchange. 4 patients suffered from relapsing disease after standard therapy with plasma exchange during the first episode. Dose schedule of Rituximab treatment consisted of 4 times 375 mg/m2 per week in parallel to continuing plasma exchange when possible. No acute severe side effects were seen after application of Rituximab. The median follow-up was 48.8 months, ranging from 10 to 98 months. All patients had an initial complete response after Rituximab treatment. At the time of re-examination, all 12 patients presented in good clinical condition, thus overall survival accounted 100%. 10 patients remained long-term disease free after initial therapy with Rituximab, two patients had recurrent disease but responded to subsequent Rituximab treatment. One patient is treated with ongoing maintenance therapy with Rituximab consisting of 375 mg/m2 every 4 weeks and remains disease free under these conditions. In our patient cohort, no long-term side effects of Rituximab treatment were noted. ADAMTS13 enzyme activity at time of re-evaluation was impaired in 50% of the patients, however no patient had an enzyme activity of less then 5%. Inhibitors against ADAMTS13 at time of re-evaluation were detected in three patients. Conclusions Rituximab is a safe and effective treatment for patients with non-familial idiopathic TTP after failure of standard therapy with plasma exchange. More than 80% of patients remain disease free during long-term follow-up, and relapses can be treated with subsequent Rituximab. Maintenance therapy is an option for frequent relapsing patients. ADAMTS13 activity might be reasonable to analyze on a regular basis to identify patients who benefit from pre-emptive Rituximab treatment. Disclosures: No relevant conflicts of interest to declare.
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