Response to Anti D immunoglobulin in Adults with Chronic Immune Thrombocytopenic Purpura in a Minority Cohort with Long Term Follow up.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4465-4465
Author(s):  
Shylendra B Sreenivasappa ◽  
Tareq Braik ◽  
Sonia Sandhu ◽  
Barbara Yim ◽  
Rosalind Catchatourian

Abstract Abstract 4465 Background Immune Thrombocytopenic Purpura (ITP) is a common hematological disorder. We sort to characterize the risk profiles and efficacy of anti D immunoglobulin in chronic ITP in a largely minority cohort. Methods 31 patients (pts) with chronic immune thrombocytopenic purpura treated 2003-2008 were studied as a retrospective cohort for clinical presentation, prognostic characteristics, time to next therapy and long term survival. Prognostic factors, time to relapse and overall survival was analyzed using fisher's exact test, logistic regression, Kaplan Meier survival analysis and Cox Proportional Hazards model. Results 31 pts 19(61.3%) female and 12(38.7%) male. 11 (35.5%) African Americans, 12(38.7%) Hispanic, 5 (16.2%) Asians and 3 (7.8%) Caucasians. Median age at diagnosis was 44 yrs (21-66). 25 (80.5%) were HIV negative and 6 (19.5%) had HIV. 16 (51.6%) had O positive blood group, 9 (29.6%) had A positive and 4 (12.9%) had B positive. The median number of co-morbidities at diagnosis was 1(0-6). Median age at which anti D immunoglobulin was initiated was 45 yrs. Pt had received a median of 3 (2-6) therapies prior to anti D therapy. All patients had received steroids, 17 (54.8%) had immunoglobulin therapy, 2 (6.5%) had vincristine and 6(19.3%) had splenectomy prior to anti D therapy. The median duration to anti D therapy was 6 months (0-228). Anti D therapy was given in the dose of 50-75mcg/kg IV weekly. Median number of doses given was 2 (1-7) doses. The response rate was 64.5%. Response was defined as Complete response, platelet count of > 100 × 109/L, Partial response >30 × 109/L. 17 (54.8%) achieved complete response, 3 (9.7%) achieved partial response. 11(35.5%) did not respond. Median time to relapse was 4 months (0-79). 9 (29%) achieved a complete response for over a year. Pt with HIV (p=0.013), O blood group (p=0.030) had a significant poor response to anti D therapy on univariate analysis. Pt with HIV (p=0.035) had poor response to therapy on multivariate analysis. O blood group (p=0.001), HIV (p=0.016), > 2 lines of therapy (p=0.007), one dose of anti D (0.037) were associated with shorter time to progression on univariate analysis. On multivariate analysis prior immunoglobulin administration (p=0.039), >2 lines of therapy (p=0.004) and single dose of anti D immunoglobulin (p=0.039) were associated with shorter time to progression. Conclusion Anti D immunoglobulin had a response rate of 64.5% which is similar to other studies. About 29% of patients had a complete response for over a year. Pts who not received anti D immunoglobulin, HIV negative and early anti D administration had the best response and longer time to progression. Disclosures: No relevant conflicts of interest to declare.

Medicina ◽  
2019 ◽  
Vol 55 (4) ◽  
pp. 112
Author(s):  
Kwiatkowska ◽  
Radkowiak ◽  
Wysocki ◽  
Torbicz ◽  
Gajewska ◽  
...  

Background and Objectives: Laparoscopic splenectomy (LS) has become the gold standard for patients with immune thrombocytopenic purpura (ITP). The total remission rate after splenectomy is 70%–90%, of which 66% is long-term. Despite this high response rate, some patients do not benefit from surgery. It is therefore important to try to identify risk factors for an unsatisfactory clinical response. The aim of this study was to assess long-term outcomes of LS for ITP and identify factors associated with increased disease remission rates. Materials and Methods: We retrospectively studied consecutive patients with ITP undergoing LS in a tertiary referral surgical center prospectively recorded in a database. Inclusion criteria were: Elective, laparoscopic splenectomy for diagnosed ITP, and complete follow-up. The cohort was divided into two groups—Group 1 (G1) patients with ITP remission after splenectomy and Group 2 (G2) patients without remission. There were 113 G1 patients and 52 G2 patients. Median follow-up was 9.5 (IQR: 5–15) years. Results: In univariate analysis, patient’s age, body mass index (BMI), preoperative platelet count, the need for platelet transfusions, and presence of hemorrhagic diathesis were shown to be statistically significant factors. Next, we built a multivariate logistic regression model using factors significant in univariate analysis. Age <41 years (odds ratio (OR) 4.49; 95% CI: 1.66–12.09), BMI <24.3 kg/m2 (OR: 4.67; 95% CI: 1.44–15.16), and preoperative platelet count ≥97 × 103/mm3 (OR: 3.50; 95% CI: 1.30–9.47) were shown to be independent prognostic factors for ITP remission after LS. Conclusion: The independent prognostic factors for ITP remission after LS revealed in our study are: age <41 years, BMI <24.3 kg/m2, and preoperative platelet count ≥97 × 103/mm3. Duration of the ITP and the time of treatment are not related to remission after LS.


Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3977-3977 ◽  
Author(s):  
Delvyn C. Case ◽  
Jacquelyn A. Hedlund ◽  
Kurt S. Ebrahim ◽  
Marjorie A. Boyd

Abstract ITP in adults is an autoimmune disease characterized by antibody-mediated thrombocytopenia. A significant number of patients relapse after initial therapy with prednisone and splenectomy when necessary, and require other therapy for continuing thrombocytopenia. Rituximab, an anti-CD20 chimeric monoclonal antibody has been utilized in ITP refractory to other modes of treatment (Feurestein, M., et al. The use of Rituximab in 28 patients with immune thrombocytopenic purpura (ITP). Proc. Am. Soc. Clin. Onc.22:187, 2003). We report on the long-term responses possible in ITP treated with Rituximab. Twenty-two patients with ITP refractory to initial conventional therapies with platelet counts &lt;30,000/ul were treated with Rituximab 375 mg/m2 IV weekly x4. Responses were characterized as partial response (PR; platelets &gt;50,000/ul but &lt;150,000/ul) and complete response (platelets &gt;150,000/ul). Patient characteristics included 5 males and 17 females with ages 24–83 years (median 58). Twenty had undergone splenectomy. Thirteen patients (59%) responded to Rituximab. Six responses were PR with durations lasting 2, 2, 3, 3, 4, and 6 months. Continuing Rituximab monthly after initial therapy of 4 weeks did not produce improved platelet counts in patients who failed Rituximab or who achieved PR. Seven responses were CR with durations of 12, 20+, 25+, 29+, 38+, 40+, and 48+ months. The one patient who relapsed at 12 months was retreated with Rituximab but did not respond. There was no correlation between response and age, sex, or duration of ITP. Neither of the patients who declined prior splenectomy responded. There were no serious complications of Rituximab infusions. Twenty-five percent of patients had mild first infusion reactions. Rituximab can produce long-term CR in 27% of patients with ITP refractory to initial therapy.


PEDIATRICS ◽  
2003 ◽  
Vol 111 (1) ◽  
pp. 87-90
Author(s):  
Derick Holt ◽  
Justin Brown ◽  
Kelly Terrill ◽  
Robert Goldsby ◽  
Rebecka L. Meyers ◽  
...  

Objective. Response to intravenous immunoglobulin (IVIG) has been shown to predict response to splenectomy in adults with immune thrombocytopenic purpura (ITP). However, reports in children have been inconsistent. We sought to determine whether response to IVIG is predictive of response to splenectomy in children. Methods. Thirty-two assessable children were identified by a retrospective chart review. Response was graded according to previously published criteria as follows: “excellent” (platelets &gt;150 000 within 1 week), “good” (platelets between 50 000 and 150 000), and “poor” (platelets &lt;50 000). “Response” refers to both splenectomy and IVIG, and response to splenectomy was counted only when it was durable. Results. Twenty-one of 23 patients who had a good or excellent response to IVIG also had an excellent response to splenectomy. Six of 9 patients who had a poor response to IVIG also had a poor response to splenectomy. Response to IVIG was a sensitive predictor of response to splenectomy in 88% of patients. Response to IVIG had a specificity of 75%, a positive predictive value of 91%, and a negative predictive value of 67%. Response to prednisone and length of time to splenectomy were not correlated with splenectomy response. Conclusions. These results suggest that response to IVIG is predictive of response to splenectomy in children with chronic ITP. This correlation may be of value in deciding whether a splenectomy should be performed in children with chronic ITP.


2020 ◽  
Vol 22 (3) ◽  
pp. 93-98
Author(s):  
V. I. Odin ◽  
N. P. Lehthlaan-Thynisson ◽  
A. D. Zolotarev ◽  
N. V. Mineeva ◽  
A. S. Polyakov

Abstract. A review of the literature on remission of immune thrombocytopenic purpura in children and a retrospective analysis of its course in a young patient who was successively applied, first to no avail, standard drug therapy, and then, due to the lack of effect, a physiotherapy technique based on the principles of activation therapy. It is known that immune thrombocytopenic purpura is a rare acquired autoimmune disease characterized by isolated thrombocytopenia with the occurrence of hemorrhagic syndrome of varying severity, which can lead to fatal bleeding. At the same time, antiplatelet antibodies are detected only in half of patients suffering from immune thrombocytopenic purpura, and therefore are not a necessary criterion for diagnosis, which in turn makes it difficult to diagnose the disease and does not allow them to be used as a criterion for the effectiveness of treatment. Despite the fact that in childhood, most cases of immune thrombocytopenic purpura occur in acute form, often with the development of spontaneous remission, but in a significant part of patients, relapses of the disease are accompanied by significant thrombocytopenia, hemorrhagic syndrome, and a decrease in the quality of life, which requires specific therapy up to splenectomy. When using the Reverse wave technique in the treatment of immune thrombocytopenic purpura, a stable, long-term remission was achieved in a young patient. The prospects of studying the application of this physiotherapy technique in clinical practice are discussed.


Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 4548-4548
Author(s):  
Nicola Cascavilla ◽  
Matteo Scaramuzzi ◽  
Michele Nobile ◽  
Matteo Dell’Olio ◽  
Antonietta Pia Falcone ◽  
...  

Abstract Background: Despite the popularity of splenectomy has decreased dramatically in the past few years, the surgical approach remains the best therapy for patients with refractory Immune Thrombocytopenic Purpura (ITP) in terms of high and durable rate of response (Vesely et al, Ann Intern Med2004; 140: 112). The recent introduction of anti-CD20 antibodies and thrombopoietins of second generation such as AMG 531 and Eltrombopag may have a relevant role (Kuter et al, Lancet2008; 371: 362) but their long-term safety and efficacy have not been still established. In parallel with new drugs, there has been an evolution in the surgery of splenectomy as well (Dolan et al, Am J Hematol2008; 83: 93). Actually, the laparoscopic surgery is considered the standard approach and the ITP represents the most common indication in 50–80% of all the laparoscopic splenectomies. Methods: The aim of this study is to evaluate the long-term complete and partial haematological response (CR + PR), as well as the short and long-term complications, of 40 patients (30 females and 10 males; median age: 38 years - range 6–71) with unresponsive ITP after one or more medical approaches and underwent laparoscopic splenectomy at our Institution from 1999 through 2006. The 40 patients accounted for 22.2% of 181 patients diagnosed in those years. An abdominal CT scan to evaluate the presence of accessory spleens was performed in all cases. All patients received meningococcal, pneumococcal and haemophilus influenzae vaccine one week before splenectomy. For 4 or 5 days before splenectomy the patients were treated with high doses of intravenous Immunoglobulins. Anti-thrombotic prophylaxis was performed with low molecular weight heparin (LMWH) for 10 days and afterwards with cardioaspirin (ASA) if the platelet count exceeded 500x10E9/L. Results: No cases required conversion to laparotomic splenectomy. An accessory spleen was found in 2 patients (5%). Immediate haematological response rate was of 100%. At date, after a median follow-up of 78 months (range 28–112 months), 36 patients (90%) remain in CR or PR with a platelet count more than 50x10E9/L and 2 patients are taking ASA. Four patients (10%) relapsed; out of which, 2 patients have a platelet count less than 10x10E9/L. Short and long-term mortality rate was 0%. Immediate postoperative complications rate was 5%: we observed 2 cases of hemoperitoneum related to a trocar’s tube and to an active bleeding, respectively, both resolved with new laparoscopic approach. The mean postoperative hospital stay was 4,5 days (range 4–8). Neither cases of bacterial sepsis in the postoperative or during the follow-up time, nor cases of splenic-portal vein thrombosis (SPVT) and no cases of neoplasms occurred. Conclusions: Our experience suggests that laparoscopic splenectomy is an excellent approach to patients with refractory ITP in terms of safety, efficacy and costs. With respect to laparotomic splenectomy, the use of laparoscopy is likely to make the splenectomy even safer and therefore suitable for a larger number of patients. Undoubtedly there is a great expectation for the new drugs (Rodeghiero et al, Am J Hematol2008; 83: 91) and we agree that only controlled comparative clinical trials (Vianelli et al, Haematologica2005; 90: 72) will be able or not to say a final word and to challenge the role of splenectomy.


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