Durability and factors associated with long term response after splenectomy for primary immune thrombocytopenia (ITP) and outcome of relapsed or refractory patients

Platelets ◽  
2010 ◽  
Vol 22 (1) ◽  
pp. 1-6 ◽  
Author(s):  
Aamer Aleem
2016 ◽  
Vol 135 (3) ◽  
pp. 162-171 ◽  
Author(s):  
Young Hoon Park ◽  
Hyeon Gyu Yi ◽  
Chul Soo Kim ◽  
Junshik Hong ◽  
Jinny Park ◽  
...  

Background: Because many physicians seem reluctant to recommend splenectomy for elderly patients with immune thrombocytopenia (ITP), we investigated the safety and efficacy of splenectomy and the predictive factors for response in these patients. Methods: 184 patients with primary ITP were retrospectively analyzed based on age at splenectomy: an elderly group (≥60 years, n = 52) and a younger group (<60 years, n = 132). Results: There was no difference in the response rate of elderly versus younger patients (80.7 vs. 80.3%, p = 0.466). Relapse (45.2 vs. 22.6%, p = 0.006), complications, and median postoperative stay (9.5 vs. 7 days, p = 0.019) were significantly higher in the elderly group. The 5-year relapse-free survival of responders was 51.8% in the elderly group and 76.3% in the younger group (p = 0.002). Response to any treatment before splenectomy (HR 2.9, 95% CI: 1.24-6.80, p = 0.014) and platelet count on postoperative day 14 ≥200 × 109/l (HR 31.43, 95% CI: 4.15-238.28, p = 0.001) were independent factors for a favorable response. Conclusions: Age ≥60 years did not influence the response to splenectomy but was associated with increased relapse and postoperative complications. Splenectomy could provide a durable long-term response for elderly ITP patients.


2013 ◽  
Vol 132 (1) ◽  
pp. 24-29 ◽  
Author(s):  
Cristina Santoro ◽  
Francesca Biondo ◽  
Erminia Baldacci ◽  
Maria Stefania De Propris ◽  
Anna Guarini ◽  
...  

2018 ◽  
Vol 22 (4) ◽  
Author(s):  
Anna Dusza ◽  
Michał Matysiak

In this article we present current investigation on primary immune thrombocytopenia in children. There are described pathomorphology, clinical symptoms, diagnosis and treatment. We also present current data from literature about genetic tests and latest data on treating options in children. Primary immune thrombocytopenia (ITP) is one of the most frequent hematological disorders in pediatric population. Although the majority of children have a self-limited and short duration of the disease. However, approximately 20-30% of those patients can develop chronic ITP, which can cause significant complications and higher mortality and reduced quality of life. Especially regarding to long-term immunosupression or surgical interventions, such like splenectomy and restrictions on daily activities to avoid trauma. Over the past decades a lot of informations has been reported about pathogenic features of ITP. Nowdays, we know that it is not only caused by increased platet destruction and decreased platet production, but also complex, multifactorial immune dysregulation, like loss of immune tolerance and generation of platelet autoantibodies. In this article we present current investigation on ITP including clinical symptoms, diagnosis, pathomorphology and latests options on treatment in children. We also present current data about genetic biomarker, such as Vanin-1 (VNN-1) which has been suggested as one of predictors of chronic disease and potentially can offer early prognosis estimation.


2013 ◽  
Vol 48 (4) ◽  
pp. 297 ◽  
Author(s):  
Laura Scaramucci ◽  
Marco Giovannini ◽  
Andrea Tendas ◽  
Pasquale Niscola ◽  
Paolo de Fabritiis

The Lancet ◽  
1991 ◽  
Vol 337 (8749) ◽  
pp. 1106 ◽  
Author(s):  
J.P. Balint ◽  
H.W. Snyder ◽  
S.K. Cochran ◽  
F.R. Jones

2017 ◽  
Vol 98 (4) ◽  
pp. 371-377 ◽  
Author(s):  
Miriam Marangon ◽  
Nicola Vianelli ◽  
Francesca Palandri ◽  
Maria Gabriella Mazzucconi ◽  
Cristina Santoro ◽  
...  

TH Open ◽  
2017 ◽  
Vol 01 (02) ◽  
pp. e73-e81
Author(s):  
Yasuyuki Arai ◽  
Hiroyuki Matsui ◽  
Tomoyasu Jo ◽  
Tadakazu Kondo ◽  
Akifumi Takaori-Kondo

AbstractCorticosteroids have been established as first-line therapy in acute primary immune thrombocytopenia (ITP), and the clinical guidelines recommend either dexamethasone (Dex) or prednisolone (PSL). The types and dosages of corticosteroids, however, have not yet been determined, because previous randomized control trials (RCTs) comparing Dex and PSL showed controversial results in terms of efficacy. To understand and interpret all available evidence, we conducted a systematic review and meta-analysis of RCTs. The main outcome measure was the incidence of sustained response (SR; platelet count >30 × 109/L for 6 months without concomitant treatments after the completion of the final therapies). Eight RCTs (totaling 704 patients) were included in this study. The incidence of SR showed no significant difference, while it was significantly higher in the Dex arm when used with posttherapy (more than one course of Dex or tapering corticosteroids added; risk ratio [RR], 1.82; 95% confidence interval [CI], 1.38–2.41; p < 0.01). A single course of Dex showed no significant difference. The overall response (platelet >30 × 109/L) at day 28 was significantly improved in the Dex arm (RR, 1.11; 95% CI, 1.01–1.22; p = 0.03) and Dex with posttherapy suppressed long-term relapse (RR of nonevent, 1.32; 95% CI, 1.10–1.59; p < 0.01). There were significantly fewer adverse events in the Dex arm (RR, 0.45; 95% CI, 0.37–0.55; p < 0.01). Use of Dex with posttherapy instead of PSL may be more beneficial as the initial therapy. Studies comparing Dex with other new strategies are essential to determine the most suitable therapeutic regimens for acute ITP.


2020 ◽  
Vol 16 (1) ◽  
pp. 61-70
Author(s):  
David O. Acero-Garcés ◽  
Herney A. García-Perdomo

Background: The first-line interventions in immune thrombocytopenia (ITP) include intravenous polyclonal immunoglobulins (IVIg), corticosteroids and anti-D immunoglobulin (anti-D). Objective: We aimed to compare the effectiveness and safety of first line treatments for newlydiagnosed primary ITP in children to increase the platelet count. Methods: We searched MEDLINE, EMBASE, LILACS and the Cochrane Central register of Controlled Trials (CENTRAL); and included the clinical trials. We performed the statistical analysis in R. Results: We included 12 studies for meta-analysis. Compared with IVIG 2g/kg, response rates were lower for prednisone 2mg/kg at 72 hours [RR 0.04 (95% CI 0.0 to 0.68)] and at 7 days [RR 0.23 (95% CI 0.08 to 0.67)]; at 48 hours, methylprednisolone 30mg/kg also showed lower response rates [RR 0.72 (95% CI 0.52 to 0.99)]. IVIG 2g/kg and 2.5g/kg had less adverse effects than Anti- D, methylprednisolone and IVIG 0.8g/kg. For rising platelet count, no statistical differences were found at 24 hours or in 7 days; at 48 hours, IVIG 2g/kg showed better results than Anti-D 75μg/kg [MD -58.84 (95% CI -87.02 to -25.66)]. After a month, platelet count with IVIG 2g/kg was higher than Anti-D 50 and 75μg/kg [-82.03 (95% CI -102.60 to -61.46) and -78.77 (95% CI -97.80 to - 59.74), respectively], but lower than methylprednisolone 50mg/kg [MD 118 (95% CI 3.88 to 232.12)]. Conclusion: The total platelet count rises higher in early and late phases with IVIG than Anti-D, but in long term it is higher with methylprednisolone. Additionally, IVIG causes less adverse effects than Anti-D and corticosteroids.


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