scholarly journals Late Central Airway Toxicity after High-Dose Radiotherapy: Clinical Outcomes and a Proposed Bronchoscopic Classification

Cancers ◽  
2021 ◽  
Vol 13 (6) ◽  
pp. 1313
Author(s):  
Juliët E. van Hoorn ◽  
Max Dahele ◽  
Johannes M. A. Daniels
Keyword(s):  
Classification System ◽  
Survival Rates ◽  
Patient Characteristics ◽  
High Dose ◽  
Vascular Changes ◽  
Significant Difference ◽  
Airway Symptoms ◽  
Suspected Recurrence ◽  
Central Airway

The study’s purpose was to identify the bronchoscopic patterns of central airway toxicity following high-dose radiotherapy or chemoradiotherapy, and to look at the consequences of these findings. Our institutional bronchoscopy database was accessed to identify main patterns of airway toxicity observed in a seven-year period. A total of 70 patients were identified with central airway toxicity, and the findings of bronchoscopy were used to derive a classification system. Patient characteristics, time from radiotherapy to toxicity, follow-up and survival were retrospectively analyzed. Results: The main bronchoscopic patterns of airway toxicity were vascular changes (telangiectasia, loss of vascularity, necrosis) and stenosis of the lumen (moderate, severe). Indications for bronchoscopy were airway symptoms (n = 28), assessment post-CRT/surgery (n = 12), (suspected) recurrence (n = 21) or assessment of radiological findings (n = 9). Stenosis was revealed by bronchoscopy at a median time of 10.0 months (IQR: 4–23.5) after radiotherapy and subsequent follow-up after identification was 23 months (IQR: 1.5–55). The corresponding findings for vascular changes were 29 months (IQR: 10.5–48.5), and follow-up after identification was nine months (IQR: 2.5–19.5). There was a statistically significant difference in survival rates between patients with necrosis and telangiectasia (p = 0.002) and loss of vascularity (p = 0.001). Eight out of 10 deceased patients with telangiectasia died of other causes and 4/8 patients with necrosis died of other causes. We identified two main patterns of central airway toxicity visualized with bronchoscopy after high-dose radiotherapy or chemoradiotherapy, and propose a bronchoscopic classification system based on these findings. Preliminary analysis suggests that the pattern and severity of radiation damage might be of prognostic value. Prospective data are required to confirm our findings.

Medulloblastoma: freedom from relapse longer than 8 years — a therapeutic cure?

1991 ◽  
Vol 75 (4) ◽  
pp. 575-582 ◽  
Author(s):  
Mark G. Belza ◽  
Sarah S. Donaldson ◽  
Gary K. Steinberg ◽  
Richard S. Cox ◽  
Philip H. Cogen
Keyword(s):  
Medical Center ◽  
Survival Rates ◽  
Adjunctive Treatment ◽  
High Dose ◽  
Content Type ◽  
Significant Difference ◽  
Long Term Follow Up ◽  
Fine Print

✓ Seventy-seven patients presenting with medulloblastoma between 1958 and 1986 were treated at Stanford University Medical Center and studied retrospectively. Multimodality therapy utilized surgical extirpation followed by megavoltage irradiation. In 15 cases chemotherapy was used as adjunctive treatment. The 10- and 15-year actuarial survival rates were both 41% with an 18-year maximum follow-up period (median 4.75 years). There were no treatment failures after 8 years of tumor-free survival. Gross total removal of tumor was achieved in 22 patients (32%); the surgical mortality rate was 3.9%. No significant difference was noted in the incidence of metastatic disease between shunted and nonshunted patients. The classical form of medulloblastoma was present in 67% of cases while the desmoplastic subtype was found in 16%. Survival rates were best for patients presenting after 1970, for those with desmoplastic tumors, and for patients receiving high-dose irradiation (≥ 5000 cGy) to the posterior fossa. Although early data on freedom from relapse suggested a possible beneficial effect from chemotherapy, long-term follow-up results showed no advantage from this modality of treatment. The patterns of relapse and survival were examined; 64% of relapses occurred within the central nervous system, and Collins' rule was applicable in 83% of cases beyond the period of risk. Although patients treated for recurrent disease could be palliated, none were long-term survivors. The study data indicate that freedom from relapse beyond 8 years from diagnosis can be considered as a cure in this disease. Long-term follow-up monitoring is essential to determine efficacy of treatment and to assess survival patterns accurately.

scholarly journals Simultaneous implant placement with autogenous onlay bone grafts: a systematic review and meta-analysis

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Guoqiang Ma ◽  
Chaoan Wu ◽  
Miaoting Shao
Keyword(s):  
Meta Analysis ◽  
Survival Rates ◽  
Bone Grafts ◽  
Implant Survival ◽  
Analysis Model ◽  
Implant Placement ◽  
Significant Difference ◽  
Delayed Implant Placement ◽  
Onlay Grafts

AbstractSeveral authors have suggested that implants can be placed simultaneously with onlay bone grafts without affecting outcomes. Therefore, the purpose of this study was to answer the following clinical questions: (1) What are the outcomes of implants placed simultaneously with autogenous onlay bone grafts? And (2) is there a difference in outcomes between simultaneous vs delayed placement of implants with autogenous onlay bone grafts? Databases of PubMed, Embase, and Google Scholar were searched up to 15 November 2020. Data on implant survival was extracted from all the included studies (single arm and comparative) to calculate point estimates with 95% confidence intervals (CI) and pooled using the DerSimonian–Laird meta-analysis model. We also compared implant survival rates between the simultaneous and delayed placement of implants with data from comparative studies. Nineteen studies were included. Five of them compared simultaneous and delayed placement of implants. Dividing the studies based on follow-up duration, the pooled survival of implant placed simultaneously with onlay grafts after <2.5 years of follow-up was 93.1% (95% CI 82.6 to 97.4%) and after 2.5–5 years was 86% (95% CI 78.6 to 91.1%). Implant survival was found to be 85.8% (95% CI 79.6 to 90.3%) with iliac crest grafts and 95.7% (95% CI 83.9 to 93.0%) with intra-oral grafts. Our results indicated no statistically significant difference in implant survival between simultaneous and delayed placement (OR 0.43, 95% 0.07, 2.49, I2=59.04%). Data on implant success and bone loss were limited. Data indicates that implants placed simultaneously with autogenous onlay grafts have a survival rate of 93.1% and 86% after a follow-up of <2.5 years and 2.5–5years respectively. A limited number of studies indicate no significant difference in implant survival between the simultaneous and delayed placement of implants with onlay bone grafts. There is a need for randomized controlled trials comparing simultaneous and delayed implant placement to provide robust evidence.

scholarly journals Partial pulpotomy without age restriction: a retrospective assessment of permanent teeth with carious pulp exposure

2021 ◽  
Author(s):  
Florin Eggmann ◽  
Thomas J. W. Gasser ◽  
Hanjo Hecker ◽  
Mauro Amato ◽  
Roland Weiger ◽  
...  
Keyword(s):  
Survival Rates ◽  
Age Groups ◽  
Permanent Teeth ◽  
Eligibility Criteria ◽  
Presumptive Diagnosis ◽  
Tooth Discoloration ◽  
Significant Difference ◽  
Pulp Exposure ◽  
The Mean

Abstract Objectives This study aimed to retrospectively evaluate clinical and radiographic outcomes of partial pulpotomy performed in permanent teeth with carious pulp exposure. Materials and methods Records of patients undergoing treatment at an undergraduate dental clinic between 2010 and 2019 were screened for partial pulpotomies in teeth with a presumptive diagnosis of normal pulp or reversible pulpitis. The follow-up had to be ≥ 1 year. Patient data were retrieved and analyzed using Mantel-Cox chi square tests and Kaplan–Meier statistics. The level of significance was set at α = 0.05. Results Partial pulpotomy was performed in 111 cases, of which 64 (58%) fulfilled the eligibility criteria. At the time of partial pulpotomy, the mean age was 37.3 (± 13.5) years (age range 18–85). The mean observation period was 3.1 (± 2.0) years. Two early failures (3.1%) and five late failures (7.7%) were recorded. The overall success rate of maintaining pulp vitality was 89.1%, with 98.4% tooth survival. The cumulative pulp survival rates of partial pulpotomy in patients aged < 30 years, between 30 and 40 years, and > 40 years were 100%, 75.5%, and 90.5%, respectively, with no significant difference between the age groups (p = 0.225). At follow-up, narrowing of the pulp canal space and tooth discoloration were observed in 10.9% and 3.1% of cases, respectively. Conclusions Across age groups, partial pulpotomy achieved favorable short and medium-term outcomes in teeth with carious pulp exposure. Clinical relevance Adequate case selection provided, partial pulpotomy is a viable operative approach to treat permanent teeth with deep carious lesions irrespective of patients’ age.

scholarly journals P1220COMPARISON OF PERITONEAL DIALYSIS CATHETER INSERTION TECHNIQUES BY NEPHROLOGISTS: SURGICAL METHOD VS BLIND METHOD USING A TROCAR

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Seok Hui Kang ◽  
Jong Won Park ◽  
Kyu Hynag Cho ◽  
Jun Young Do
Keyword(s):  
Peritoneal Dialysis ◽  
Survival Rates ◽  
Peritoneal Dialysis Catheter ◽  
Dialysis Catheter ◽  
Patient Death ◽  
Surgical Method ◽  
Surgical Methods ◽  
Significant Difference ◽  
Insertion Techniques

Abstract Background and Aims Successful PD requires timely peritoneal dialysis catheter (PDC) insertion and management of PDC related complications. Some societies have recently made an effort to increase PD and PDC insertion by nephrologists is an important issue. The aim of the present study was to evaluate comparative analysis of PDC insertion between blind and surgical methods by nephrologists. Method We reviewed medical records at a tertiary medical center in Korea and identified 249 adults who underwent first-time PDC insertion. All PDC insertions were performed using the blind or surgical methods. In our hospital during study period, two of three nephrologists performed the blind method routinely in all of eligible patients (blind group, n = 144). One of three nephrologists performed the surgical method in all of eligible patients (surgical group, n = 105). During follow-up, we collected data regarding peritoneal dialysis peritonitis (PDP), exit site and/or tunnel infection (ESI/TI). Catheter survivor was defined as maintaining of PD at July 2019 or PDC removal by PDC unrelated problems such as patient death due to PDC unrelated factors, kidney transplantation, patient demand, inadequate PD, improved renal function, poor oral intake due to abdominal distension, and colon cancer. Catheter non-survivor and/or PDC associated removal was defined as PDC removal by PDC related problems such as PDP, ESI/TI or PDC malfunction. Intervention-free non-survivor was defined as PDC revision, removal, or exchange by PDC related problems. Results Mean age at the PDC insertion in blind and surgical groups were 57.5 ± 13.7 and 56.3 ± 12.9 years, respectively (P = 0.640). There were no significant differences in age, sex, body mass index, underlying disease of ESRD, and Davies comorbidity index between the 2 groups. Mean follow-up durations were 37.0 ± 26.3 and 32.6 ± 23.4 months in the blind and surgical groups, respectively (P = 0.172). Total numbers of patients with one or more PDP events during follow-up period were 72 (50.0%) and 42 (40.0%) in blind and surgical groups (P = 0.118). Total numbers of PDP episodes were 157 and 100, respectively. Total numbers of patients with one or more ESI/TI events during follow-up period were 14 (9.7%) and 7 (6.7%) in blind and surgical groups (P = 0.392). Total numbers of ESI/TI episodes were 27 and 8, respectively. The 5-year PDC survival rates were 87.0% and 91.1% in the blind and surgical groups, respectively (P = 0.995, Figure 1). The 5-year intervention-free survival rates were 79.6% and 77.0% in the blind and surgical groups, respectively (P = 0.723). The leading cause of PDC removal was patient death. There was no significant difference in the distributions of cause of PDC removal in the 2 groups (P = 0.335). PDC associated removal rates in blind and surgical groups were 14 (18.4%) and 9 (16.4%), respectively (P = 0.760). Conclusion Our study shows that catheter outcomes including infectious and mechanical complications and catheter survival are similar between blind and surgical insertion techniques by nephrologists.

P3453Gender difference in impact of ischemic heart disease on long-term outcome in patients with heart failure reduced ejection fraction

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
H J Kim ◽  
M A Kim ◽  
D I Lee ◽  
H L Kim ◽  
D J Choi ◽  
...  
Keyword(s):  
Heart Failure ◽  
Clinical Outcomes ◽  
Survival Rates ◽  
Free Survival ◽  
Reduced Ejection Fraction ◽  
Clinical Events ◽  
Significant Difference ◽  
Patients With Heart Failure

Abstract Background Ischemic heart disease (IHD) is a major underlying etiology in patients with heart failure (HF). Although the impact of IHD on HF is evolving, there is a lack of understanding of how IHD affects long-term clinical outcomes and uncertainty about the role of IHD in determining the risk of clinical outcomes by gender. Purpose This study aims to evaluate the gender difference in impact of IHD on long-term clinical outcomes in patients with heart failure reduced ejection fraction (HFrEF). Methods Study data were obtained from the nationwide registry which is a prospective multicenter cohort and included patients who were hospitalized for HF composed of 3,200 patients. A total of 1,638 patients with HFrEF were classified into gender (women 704 and men 934). The primary outcome was all-cause death during follow-up and the composite clinical events of all-cause death and HF readmission during follow-up were also obtained. HF readmission was defined as re-hospitalization because of HF exacerbation. Results 133 women (18.9%) were died and 168 men (18.0%) were died during follow-up (median 489 days; inter-quartile range, 162–947 days). As underlying cause of HF, IHD did not show significant difference between genders. Women with HFrEF combined with IHD had significantly lower cumulative survival rate than women without IHD at long-term follow-up (74.8% vs. 84.9%, Log Rank p=0.001, Figure 1). However, men with HFrEF combined with IHD had no significant difference in survival rate compared with men without IHD (79.3% vs. 83.8%, Log Rank p=0.067). After adjustment for confounding factors, Cox regression analysis showed that IHD had a 1.43-fold increased risk for all-cause mortality independently only in women. (odds ratio 1.43, 95% confidence interval 1.058–1.929, p=0.020). On the contrary to the death-free survival rates, there were significant differences in composite clinical events-free survival rates between patients with HFrEF combined with IHD and HFrEF without IHD in both genders. Figure 1 Conclusions IHD as predisposing cause of HF was an important risk factor for long-term mortality in women with HFrEF. Clinician need to aware of gender-based characteristics in patients with HF and should manage and monitor them appropriately and gender-specifically. Women with HF caused by IHD also should be treated more meticulously to avoid a poor prognosis. Acknowledgement/Funding None

scholarly journals Liver Transplantation from Voluntary Organ Donor System in China: A Comparison between DBD and DCD Liver Transplants

2019 ◽  
Vol 2019 ◽  
pp. 1-7
Author(s):  
Wei Chen ◽  
Dipesh Kumar Yadav ◽  
Xueli Bai ◽  
Jianying Lou ◽  
Risheng Que ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Organ Donation ◽  
Survival Rates ◽  
Organ Donor ◽  
Long Term Outcomes ◽  
Significant Difference ◽  
Short And Long Term ◽  
Donor System

Background. In China, the cases of liver transplantation (LT) from donation after citizens’ death have rose year by year since the citizen-based voluntary organ donor system was initiated in 2010. The objective of our research was to investigate the early postoperative and late long-term outcomes of LT from donation after brain death (DBD) and donation after circulatory death (DCD) according to the current organ donation system in China. Methods. Sixty-two consecutive cases of LT from donation after citizens’ death performed in our hospital between February 2012 and June 2017 were examined retrospectively for short- and long-term outcomes. These included 35 DCD LT and 27 DBD LT. Result. Subsequent median follow-up time of 19 months and 1- and 3-year graft survival rates were comparative between the DBD group and the DCD group (81.5% and 66.7% versus 67.1% and 59.7%; P=0.550), as were patient survival rates (85.2% and 68.7% versus 72.2% and 63.9%; P=0.358). The duration of ICU stay of recipients was significantly shorter in the DBD group, in comparison with that of the DCD group (1 versus 3 days, P=0.001). Severe complication incidence (≥grade III) after transplantation was identical among the DBD and DCD groups (48.1% versus 60%, P=0.352). There was no significant difference in postoperative mortality between the DBD and DCD groups (3 of 27 cases versus 5 of 35 cases). Twenty-one grafts (33.8%) were lost and 18 recipients (29.0%) were dead till the time of follow-up. Malignancy recurrence was the most prevalent reason for patient death (38.8%). There was no significant difference in incidence of biliary stenosis between the DBD and DCD groups (5 of 27 cases versus 6 of 35 cases, P=0.846). Conclusion. Although the sample size was small to some extent, this single-center study first reported that LT from DCD donors showed similar short- and long-term outcomes with DBD donors and justified the widespread implementation of voluntary citizen-based deceased organ donation in China. However, the results should be verified with a multicenter larger study.

scholarly journals Comparison of Survival Rates of Stainless-Steel Crowns Placed with and without Pulpotomy: A Two-Year Retrospective Study

2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
Fatmah N. AlMotawah ◽  
Sharat Chandra Pani ◽  
Tala AlKharashi ◽  
Saleh AlKhalaf ◽  
Mohammed AlKhathlan ◽  
...  
Keyword(s):  
Stainless Steel ◽  
General Anesthesia ◽  
Survival Rates ◽  
Survival Outcome ◽  
Survival Outcomes ◽  
Proximal Caries ◽  
Kaplan Meier ◽  
Significant Difference ◽  
Chi Squared

Aim. This study aimed to retrospectively compare the survival outcomes over two years between teeth with proximal dental caries that were restored with stainless-steel crowns to those that were pulpotomized and then restored with a stainless-steel crown in patients who were rehabilitated under general anesthesia. Participants and Methods. The records of 131 patients aged between two to six years who had stainless-steel crowns placed under general anesthesia and had two-year follow-up were screened. 340 teeth with moderate proximal caries on the radiograph (D2) were included in the study. Of these, 164 teeth were treated with a pulpotomy and stainless-steel crown, while 176 teeth were crowned without a pulpotomy. The type of each tooth was compared using the Chi-squared test and Kaplan–Meier survival analysis, and curves were plotted based on the two-year outcomes. Results. Treatment: the sample comprised 59 males (mean age 4.73 years, SD ± 1.4 years) and 72 females (mean age 5.2 years, SD ± 2.0 years). The Kaplan–Meier regression model showed no significant difference in survival outcomes between teeth that had been pulpotomized and those that had not ( p  = 0.283). Conclusion. Within the limitations of the current study, we can conclude that performing a pulpotomy does not influence the survival outcome of mild/moderate proximal caries restored with stainless-steel crowns under general anesthesia.

Long-Term Follow-Up of a Randomized Phase III Multicenter Study Comparing a Standard Versus an Intensified Conditioning Regimen for High-Dose Chemotherapy in Patients with Multiple Myeloma.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 945-945
Author(s):  
Roland Fenk ◽  
Peter Schneider ◽  
Martin Kropff ◽  
Ali-Nuri Huenerlituerkoglu ◽  
Ulrich Steidl ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Stem Cell ◽  
Conditioning Regimen ◽  
High Dose Chemotherapy ◽  
Phase Iii ◽  
Study Endpoint ◽  
Primary Study ◽  
High Dose ◽  
Significant Difference

Abstract High-dose chemotherapy (HDT) improves the outcome of patients with multiple myeloma (MM) in comparison to conventional chemotherapy. Dose-escalating strategies including tandem HDT are currently evaluated to further improve remission rates and survival of patients. Therefore we conducted a randomized multicenter trial to compare an intensified conditioning regimen with the current standard high-dose melphalan. The primary study endpoint was response rate, with overall survival (OS), event-free survival (EFS) and toxicity analysed as secondary endpoints. Between 1997 and 1999 a total of 56 patients with stage II and III disease, who were matched for age (median 56 years), number of previous therapies (median time from diagnosis to transplant 7 months) and different risk factors (beta2-microglobulin, LDH, CRP, cytogentic abnormalities, chemoresistant disease, IgA-subtype, renal impairment), were randomized. All patients received 2 courses of oral idarubicine/dexamethasone and 2 courses of intravenous cyclophosphamide/adriamycine in combination with G-CSF followed by peripheral stem cell collection. Thirty patients were treated with melphalan 200mg/m2 (HD-M) whereas 26 patients received idarubicine 42mg/m2, melphalan 200mg/m2 and cyclophosphamide 120mg/kg (HD-IMC) followed by autologous blood stem cell transplantation. Acute toxicity was higher with HD-IMC, including 5 (20%) treatment-related deaths due to infections versus none (0%) in the HD-M group. This lead to early termination of the study. Severity of mucositis (grade III-IV 19 vs. 8 pts., p=0.001), CRP (20 vs. 7 mg/dl, p<0.001), days of fever (11 vs. 3, p<0.001), days with iv-antibiotics (13 vs. 4, p<0.001), number of erythrocyte-transfusions (6 vs. 2, p<0.001), number of platelet-transfusions (16 vs. 4, p<0.001) and days to granulocyte engraftment (18 vs. 11, p=0.007) were significantly higher after HD-IMC. After a follow-up of 5 years analysis restricted to patients surviving the first 30 days after HDT showed a trend to higher response rates (CR+vgPR: 47% (95%CI 24–72%) vs. 35% (95%CI 18–56%), PR 37% (95%CI 17–63%) vs. 48% (95%CI 29–68%) and time-to-progression (median 31 vs. 15 months, p=0.1) in the HD-IMC treatment arm in comparison to HD-M, but there was no significant difference in EFS and OS (median 22 vs. 30, p= 0.31 and 66 vs. 66 months, p=0.8, respectively). Univariate analysis demonstrated that LDH levels > 200 U/L (p=0.04) and chemoresistant disease (p=0.05) were a bad prognostic factor for EFS. Beta2-Microglobulin levels > 5mg/dl (p=0.01), abnormal conventional cytogenetics (p=0.02) and LDH levels > 200 U/L (p=0.03) were predictive for an inferior OS. In conclusion intensified conditioning for HDT had an intolerable high treatment-related mortality and did not improve EFS and OS in patients with multiple myeloma.

The Utility of Consolidative Upfront High Dose Chemoradiotherapy and ASCT in Patients with Mantle Cell Lymphoma (MCL).

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 2072-2072
Author(s):  
Daniel Persky ◽  
Carol S. Portlock ◽  
Simone Lessac-Chenen ◽  
Alexia Iasonos ◽  
Andrew D. Zelenetz ◽  
...  
Keyword(s):  
Bone Marrow Involvement ◽  
Conditioning Regimen ◽  
Stage Iv ◽  
Progression Free Survival ◽  
High Dose Chemotherapy ◽  
High Dose ◽  
Significant Difference ◽  
Dose Dense

Abstract Introduction: Two approaches to improve progression-free survival (PFS) in MCL are intensifying induction, as with hyperCVAD/M-A regimen, or intensifying consolidation with high dose chemoradiotherapy (HDT) and ASCT as in the prospective European MCL Network Trial. At MSKCC the strategy is to incorporate both approaches by administering an anthracycline-containing regimen in a dose dense fashion (CHOP- or R-CHOP-14) followed by consolidation with ICE (ifosfamide, carboplatin, etoposide) and HDT/ASCT. Patients: Forty six patients with newly diagnosed MCL underwent HDT/ASCT between 11/96 and 2/05. The median age was 55 years; 74% were male; 72% had bone marrow involvement, 39% had GI involvement, 7% were in leukemic phase, and 91% presented with stage IV disease. Splenomegaly was seen in 35%, B symptoms in 9%, KPS>70 in 93%, elevated LDH in 23%, and blastoid histology in 9%. Results: Induction was 4 to 6 cycles of CHOP-14 (43%), R-CHOP-14 (37%), or other doxorubicin-containing regimen (20%). Consolidation was performed with 2–3 cycles of ICE in 53% or R-ICE in 39%. Upfront treatment was well tolerated and permitted adequate stem cell collection and prompt transition to HDT/ASCT. Conditioning regimens were TBI/CY/VP-16 (59%) and BEAM (41%). Involved field radiation therapy was administered to 65%. Post-ASCT rituximab maintenance was given to 39%, with 57% of patients receiving rituximab as part of their treatment. Anthracycline-based induction led to CRu of 44% and ORR of 98%. Seventy two percent of patients were transplanted in CR, while the remaining 28% were in PR. At a median follow-up of 2.5 years (range 0.4–8.0 years) 17% of the patients have died and 24% have had progressive disease. The median OS and PFS have not been reached (lower 95% CI, 5.7 years and 4.4 years, respectively). The 5-year PFS and OS are 58% and 83%, respectively. The use of rituximab at any point during treatment prolonged PFS - only 1 of 26 patients receiving rituximab relapsed, as compared to 10 of 20 patients who were rituximab naive (p=0.03); thus far there is no significant difference in OS. There was no day 100 treatment related mortality. One patient developed bronchiolitis obliterans after ASCT and died of pulmonary fibrosis 6.5 years later; 3 patients have died of secondary cancers - one case each of MDS (1.6 years after ASCT), melanoma and lung cancer. Conclusion: These data provide evidence that dose-dense induction with CHOP-14 or R-CHOP-14 and consolidation with ICE/HDT/ASCT appears to be safe and effective, with minimal acute toxicity. Although the median follow-up is short, the use of rituximab appears to improve PFS. This contrasts with the findings of German LGLSG and may be a consequence of in vivo rituximab purging. Future therapy could incorporate all the successful elements of prior treatment programs, including R-CHOP-14, R-ICE, and radioimmunotherapy with high dose chemotherapy conditioning regimen, followed by ASCT and rituximab-based maintenance. PFS stratified by Rituximab PFS stratified by Rituximab

Myeloproliferative Disease in the Pathogenesis and Survival of Budd-Chiari Syndrome.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 1480-1480
Author(s):  
Jasper H. Smalberg ◽  
Sarwa D. Murad ◽  
Eric Braakman ◽  
Peter Valk ◽  
Mies C. Kappers-Klunne ◽  
...  
Keyword(s):  
Mutation Analysis ◽  
Survival Rates ◽  
Vena Cava ◽  
Aetiological Factor ◽  
Budd Chiari Syndrome ◽  
Jak2 Mutation ◽  
Who Criteria ◽  
Significant Difference ◽  
Chiari Syndrome

Abstract Budd-Chiari syndrome (BCS) is a rare disorder caused by obstruction of the hepatic veins or the suprahepatic inferior vena cava. Myeloproliferative diseases (MPD) are the most important aetiological factor in BCS. The aim of this study was to evaluate multifactorial aetiology in BCS patients with MPD, to assess potential added value of the recently discovered JAK2 mutation in the diagnostics of MPD in BCS, and to determine the survival of MPD patients in BCS. All patients referred to our university hospital with primary, non-malignant BCS between January 1980 and January 2006 were included in this study (n=40). Median age was 28.4 years (18.4–53.3), 26 patients (65%) were female and in nine patients (23%) additional portal vein thrombosis (PVT) was present. Overall mean follow-up was 7.1 ± 6.9 years. Only two patients were lost to follow-up, of which one MPD patient. In addition to standard MPD work-up according to WHO criteria, JAK2 mutation analysis was performed in 17 patients. MPD was present in 33% of the patients: Polycythemia Vera (n=6), Essential Thrombocythemia (ET) (n=6) and unclassifiable MPD (n=1). JAK2 mutation was identified in seven out of the 17 tested BCS patients (41%). In two patients suspect for ET, but who failed to meet WHO criteria, JAK2 mutation analysis led to the diagnosis of MPD. In 38% of patients with MPD additional pro-thrombotic factors were present. Survival rates in patients with MPD at 1, 5, and 10 years remained constant at 92% (95% CI, 78%–100%). Survival rates in patients without MPD at 1, 5, and 10 years were 89% (95% CI, 77%–100%), 64% (95% CI, 44%–85%) and 53% (95% CI, 28%–79%), respectively. There was no significant difference in survival between the two (P=0.18). Additional PVT was only present in patients without MPD in our population, which may account for the slightly lowered survival of these patients, since more extensive thrombosis in the splanchnic area is associated with poorer survival. In addition, three patients with and one patients without MPD underwent liver transplantation, which may have affected survival rate in favour of MPD patients. In conclusion, we report MPD in 33% of our BCS patients. In 38% of the patients with MPD, additional pro-thrombotic factors were present. These results enforce the necessity of extensive screening for additional pro-thrombotic conditions. Our study clearly confirms the importance of JAK2 mutation analysis in patients with BCS. Long term follow-up did not show a significant difference in survival between patients with and without MPD.

Sign in / Sign up

Export Citation Format

Share Document