Long-Term Favorable Results by Arteriovenous Graft with Omniflow II Prosthesis for Hemodialysis

&lt;i&gt;Aims and Methods:&lt;/i&gt; In order to decrease arteriovenous graft (AVG) failure and improve long-term patency, we used Omniflow II to perform AVG for hemodialysis access in 38 patients with very compromised vessels who were not suitable for other forms of AVG. &lt;i&gt;Results:&lt;/i&gt; At a median follow-up of 38 (range 6–55) months, 31/38 (81%) patients were still alive. At 6, 12 ,18 and 24 months, primary patency was 92, 80, 68 and 60%, whereas secondary patency was 83, 80, 78 and 75%, respectively. The cumulative 38-month prosthetic AVG patency was 70%. No infective event related to the vascular prosthesis occurred. Neither AVG thrombosis nor modifications in thrombophilic pattern were observed; these findings confirm the high hemocompatibility of this prosthetic vascular device. &lt;i&gt;Conclusion:&lt;/i&gt; Our experience is extremely encouraging for the use of new biosynthetic devices such as Omniflow II.</jats:p

Health-Related Quality of Life in Patients with Primary Immune Thrombocytopenia (pITP): Investigating Differences Amongst Newly Diagnosed, Persistent and Chronic Pitp Patients

Abstract Introduction: Very little is known about health-related quality of life (HRQOL) of patients diagnosed with primary Immune Thrombocytopenia (pITP). Also, the paucity of available data mainly stems from patients enrolled in randomized controlled trials thus limiting generalizability of findings to patients routinely seen in real world practice. Aim: The main objective of this study is to compare HRQOL of pITP patients with that of their peers from the general population and to investigate whether HRQOL differences exist among those classified as (Rodeghiero et al, Blood. 2009 Mar 12;113(11):2386-93): newly diagnosed, persistent and chronic pITP patients. Methods: Adult patients with pITP were consecutively enrolled, regardless of therapy, in 25 centers. All patients were invited by their treating physicians in the hospital. Consenting patients were requested to complete a battery of patient-reported outcome (PRO) questionnaires. The primary HRQOL outcome measure was the Medical Outcomes Study 36-Item Short-Form Health Survey (SF-36). It consists of 36 items covering eight generic health status/QoL domains: physical functioning (PF), role limitations due to physical health (RP), bodily pain (BP), general health perceptions (GH), vitality (VT), social functioning (SF), role limitations due to emotional problems (RE), and mental health (MH). To minimize possible bias in comparisons of pITP patients HRQOL outcomes to general population norms, a propensity score matching approach plus further regression adjustment was adopted to select the best possible case-control pairs. The group of potential controls was a representative sample of 1997 adults without the disease from a previous study which provided SF-36 population norms. Controls from these data were matched to pITP patients by an optimal nearest-neighbour matching algorithm based on age, sex, education, and geographic area. Multiple linear regression analyses were performed on the matched dataset to compute adjusted mean differences (α=0.05), using selected a priori key HRQOL confounders as covariates: age, sex, education, geographic area, and marital status. Effect sizes were computed and clinical significance of mean scores SF-36 differences was also estimated. Results: Out of 424 patients invited to participate, 420 returned the questionnaires (99% compliance). At study participation, mean age of patients was 54 years (range 18-89) with the majority being women (N=264, 64%). The mean platelet count at diagnosis was 27.6 x109/L and the mean platelet count at last medical visit, before the HRQOL assessment, was 119 x109/L. Two-hundred twenty patients (50%) had at least 1 comorbidity. At the time of HRQOL assessment there were 82%, 64% and 44% of patients receiving active treatments, respectively in the newly diagnosed, persistent and chronic pITP group. Splenectomy was performed in 8% and 22% of the persistent and chronic pITP patients, respectively. No statistically significant differences existed among the three disease groups with regard to age, gender and presence of comorbidity. When compared with their peers in the general population, worse statistically significant outcomes (P&lt;0.05) were found for five out of the eight SF-36 scales. The largest clinically meaningful difference (Δ=16 points) was found for the RE scale. Comparison by disease group (each of which versus their respective peers in the general population), revealed greater HRQOL impairments in persistent pITP patients across all physical and mental health HRQOL domains. Differences between newly diagnosed and chronic pITP patients with their peers in the general populations were marginal. However, for persistent pITP patients medium to large impairments were found when compared with their peers in the following domains: PF (ES=0.87, P=0.001), RP (ES=0.93, P&lt;0.001), and SF (ES=0.78, P=0.004), RE (ES=0.74, P=0.004), and MH (ES=0.55, P=0.026). Conclusions: Persistent pITP patients are those experiencing the most important limitations in both physical and mental HRQOL domains with respect to their peers from the general population. This information can help physicians to pay special attention to this patient group in their daily clinical practice. Disclosures Gaidano: Celgene: Research Funding; MorphoSys; Roche; Novartis; GlaxoSmithKline; Amgen; Janssen; Karyopharm: Honoraria, Other: Advisory boards. Rodeghiero:Celgene Corporation: Honoraria, Research Funding. </jats:sec

Health-related quality of life and burden of fatigue in patients with primary immune thrombocytopenia by phase of disease

The main objective of this study was to compare health-related quality of life (HRQOL) of primary immune thrombocytopenia (pITP) patients with that of general population, overall, and by patient group (i.e., newly diagnosed, persistent, and chronic patients). Fatigue was also investigated as a secondary objective. Overall, 424 adult patients were enrolled in a multicenter observational study and the control group consisted of a representative sample from the general population. Propensity score matching plus further multivariate linear regression adjustment was used to compare HRQOL outcomes between pITP patients and general population. Mean age of patients was 54 years. Of those with HRQOL assessment, 99 patients (23.6%) were newly diagnosed, 53 (12.6%) were persistent, and 268 (63.8%) were chronic pITP patients. Comparison by patient group versus their respective peers in the general population revealed greater impairments in persistent pITP patients. Persistent pITP patients reported clinically meaningful impairments in physical functioning (-15; 95% CI -24.1 to -5.8; P = 0.002), social functioning (-15.3; 95% CI -25.5 to -5.1; P = 0.004), role physical (-28.4; 95% CI -43.1 to -13.7; P < 0.001), role emotional (-23.9; 95% CI -40.1 to -7.7; P = 0.004), and mental health scales (-11.3; 95% CI -21.2 to -1.4; P = 0.026) of the SF-36 questionnaire. Higher fatigue severity was associated with lower physical and mental HRQOL outcomes. Our findings suggest that the burden of the disease and treatment might depend on the disease phase and that persistent pITP patients are the most vulnerable subgroup. Am. J. Hematol., 2016. © 2016 Wiley Periodicals, Inc

Predictors of Outcome and Response to Therapy in Primary Autoimmune Hemolytic Anemia: A Gimema Study of 307 Patients

Abstract Autoimmune hemolytic anemia (AIHA) is a greatly heterogeneous condition both in terms of clinical presentation and response to treatment, usually classified as warm (WAIHA), cold (CAD), mixed, and atypical forms. The aim of this study was to identify predictors of outcome and response to therapy considering in particular the serological characteristics and the severity of anemia at onset. We evaluatedretrospectively 307 patients (112 M and 195 F, median age at diagnosis 63, range 1-97), diagnosed between 1978 and 2013 and followed-up for a median of 33 months (range 12-372); 60% of cases were WAIHA, 27% CAD, 8% mixed, and 5% atypical (14 DAT- and 1 DAT+ for IgA only). Hemoglobin values were lower in mixed (median 5.8, range 2-10.7 g/dL) atypical (6.2, 3-9), and in IgG+C3 DAT+ WAIHA (6.9, 2.9-11.5). Twenty-one subjects were diagnosed with Evans’ syndrome, the majority of them WAIHA, with a severe onset. Considering anemia at onset, 27% of cases had Hb levels &lt;6, 36% Hb 6.1-8, 24% Hb 8.1-10, and 13% Hb&gt;10 g/dL; the most severe cases were mainly mixed and atypical forms (P=0.0001). Regarding therapy, 47% of cases were treated with one therapy line only, 26% with two, 13% with three, and 4% with four or more lines. Sixty % of WAIHA received first line steroid therapy only, 20 CAD required no treatment, and patients with IgG+C DAT+ WAIHA, mixed, and atypical forms were more frequently treated with 2 or more therapy lines (P&lt;0.0001); the gender- and age-adjusted cumulative incidence of relapse was significantly increased in more severe cases by Fine and Gray model (Figure). Response to steroids was observed in ~75% of cases, with lower rates in CAD and generally observed at high steroid dosages. Splenectomy (32 cases, mostly WAIHA or severe forms) had a response rate of 75%, but was ineffective in 2/3 CAD; the relapse rate was 8/24 (33%) after a median of 41 months. Regarding immunosuppressants (31 cases azathioprine, 40 cyclophosphamide, and 12 cyclosporine) the OR was 50-70% (PR 20-40), irrespective of serological type and severity of anemia, although the simultaneous administration of steroid in most cases may weaken these results; the relapse rate was 8/60 (13%) after a median of 11 months. Rituximab (55 cases at conventional, and in 19 at low doses (LD) of 100 mg /weekly x 4) had an 80% OR (35% PR). Predictors of response to LD were WAIHA, younger age, and shorter interval between diagnosis and rituximab therapy; at variance, OR to conventional doses occurred irrespectively of age, serological type, clinical severity at onset, and disease duration. The relapse rate was 5% (2/42, of whom 1 CAD) for standard and 38% (6/16, of whom 5 CAD) for LD, and relapses occurred mostly within the first year after treatment. As regards complications, infections occurred in 26 cases (10 grade 3, 11 grade 4, and 5 grade 5), irrespective of serological AIHA type and severity at onset, and of the number of therapy lines; on the contrary, they were observed more frequently in splenectomized cases. Acute renal failure was recorded in 6 cases and was not associated with AIHA clinical or serological characteristics. A thrombotic event was recorded in 11% and was associated with severe onset, higher median LDH levels, and previous splenectomy. At the time of the analysis 63 cases (21%) have died, of whom 11 because of AIHA (3.6%); death was not associated with the severity of anemia at onset, nor with the serological type of AIHA; at variance, it was associated with infections (HR 11.47, 95% CI 3.43-38.4, p=0.0004), acute renal failure (HR 17.99, 95% CI 4.73-68.40, p=0.001), Evans’ syndrome (HR 6.8, 95% CI 1.99-23.63, P=0.0074), previous splenectomy (HR 3.21, 95% CI 0.92-11.25), and multi-treatment (4 or more lines of therapy; HR 9.1, 95% CI 2.41-34.36, p=0.0076). Death was not associated with thrombotic events, nor with the type of treatment, in particular immunosuppressants or rituximab. In conclusion, we showed that AIHA cases with a severe onset, mostly mixed and atypical forms, are frequently refractory to different therapies. Although obtained retrospectively, our results suggest to put forward rituximab among second line options, given its efficacy and safety. In addition, standard rituximab doses should be preferred in CAD, whereas lower doses may be equally effective in WAIHA and mixed forms. Finally, we suggest to defer splenectomy after rituximab, given the increased risk of thromboembolism, infections and fatal outcome in splenectomized patients. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare. </jats:sec