Characteristics and Survival of 29 Patients with Sezary Syndrome.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 5587-5587
Author(s):  
Francesca Sampogna ◽  
Marina Frontani ◽  
Giannandrea Baliva ◽  
Damiano Abeni ◽  
Giuseppe A. Lombardo ◽  
...  

Abstract We created a relational database of patients with cutaneous T-cell lymphoma (CTCL) to collect in a standardised fashion, anagraphic variables, clinical history, clinical, histological, haematological, and immunological information on CTCL patients hospitalised at IDI-IRCCS, Rome, Italy. At present, there are data on 424 patients, hospitalised from 1983 to July 2005. Active follow-up is performed yearly to ensure a standardised ascertainment of survival time. For deceased patients, the actual date of death (for all causes) is recorded, while surviving patients are censored at the date of last contact. The database includes 29 patients with Sezary syndrome (SS). Follow-up times ranged from 0 to 105 months. At first hospitalisation the median values of cells/mL were: white blood cells (WBC) 8750, neutrophils 4250, eosinophils 140, basophils 120, lymphocytes 2760, monocytes 500, CD3+ 2780, CD4+ 2431, CD8+ 192, CD19+ 96. Seventeen patients were deceased. We included in the Kaplan-Meier survival analysis only patients who were diagnosed before July 2004 (n=26). Median survival time from diagnosis was 52 months. No significant differences were observed in mortality for WBC (cutoff 9000 cells/uL), neutrophils (cutoff 4500 cells/uL), basophils (cutoff 200 cells/uL), lymphocytes (cutoff 3000 cells/uL), monocytes (cutoff 500 cells/uL), CD3+ (cutoff 2000 cells/uL), CD4+ (cutoff 2000 cells/uL), CD8+ (cutoff 200 cells/uL), CD19+ (cutoff 70 cells/uL). A lower survival was observed for patients with eosinophils <200 cells/uL (p=.08). Survival in patients with SS does not seem to be influenced by haemathologic parameters. However, patients with long-term survival (>90 months) are observed, and their characteristics should be further investigated. Survival analysis of 26 patients with Sézary syndrome, Rome, Italy, 1991–2004. Survival analysis of 26 patients with Sézary syndrome, Rome, Italy, 1991–2004.

2018 ◽  
Vol 2018 ◽  
pp. 1-7 ◽  
Author(s):  
Mostafa Alavi-Moghaddam ◽  
Mohammad Chehrazi ◽  
Shamila D. Alipoor ◽  
Maryam Mohammadi ◽  
Alireza Baratloo ◽  
...  

Introduction. miRNAs contribute to a variety of essential biological processes including development, proliferation, differentiation, and apoptosis. Circulating microRNAs are very stable and have shown potential as biomarkers of cardiovascular disease. microRNA-208b expression was increased in the blood of patients with acute myocardial infarction (AMI) and has been proposed as a biomarker for early diagnosis. In this pilot study, we investigate the potential of circulating miR-208b as a prognostic biomarker of 6-month survival in AMI patients. Methods. Plasma samples from 21 patients and 8 age- and gender-matched healthy adults were collected, and circulating levels of miR-208b were detected using quantitative real-time PCR. Results. miR-208b levels were higher in healthy control subjects (9.6-fold; P≤0.05). Within the AMI patients, the levels of miR-208b were significantly lower in the survivor versus nonsurvivor group (fold change = 6.51 and 14.1, resp.; P≤0.05). The Kaplan-Meier curve revealed that the 6-month survival time was significantly higher among AMI patients with a relative expression of miR-208b lower than 12.38. The hazard ratio (HR) for the relative expression of miR-208b (<12.38 was the reference) was 5.08 (95% CI: 1.13–22.82; P=0.03). Conclusion. Our results showed that elevated miR-208b expression was associated with reduced long-term survival in AMI patients. These pilot data indicate the need for a large follow-up study to confirm whether miR-208b can be used as a predictor of 6-month survival time after AMI.


2019 ◽  
Vol 45 (2) ◽  
pp. 160-166 ◽  
Author(s):  
Farhad Farzaliyev ◽  
Hans-Ulrich Steinau ◽  
Halil-Ibrahim Karadag ◽  
Alexander Touma ◽  
Lars Erik Podleska

In this retrospective study, we analysed the long-term oncological and functional results after extended ray resection for sarcoma of the hand. Recurrence-free and overall survivals were calculated using the Kaplan–Meier method. The function of the operated hand was assessed with the Michigan Hand Questionnaire and compared with the contralateral side. Extended ray resection was performed in 25 out of 168 consecutive patients with soft-tissue and bony sarcomas of the hand. The overall 5- and 10-year, disease-specific survival rates were 86% and 81%, respectively. Local recurrences were observed in two patients. The Michigan Hand Questionnaire score for the affected hand at follow-up in nine patients was 82 points versus 95 for the healthy contralateral hands. We conclude that extended ray resection of osseous sarcomas breaking through the bone into the soft tissue or for soft tissue sarcomas invading bone is a preferable alternative to hand ablation when excision can be achieved with tumour-free margins. Level of evidence: III


2019 ◽  
Vol 21 (Supplement_6) ◽  
pp. vi285-vi285
Author(s):  
Martin van den Bent ◽  
Khe Hoang-Xuan ◽  
Alba Brandes ◽  
Johan Kros ◽  
M C M Kouwenhoven ◽  
...  

Abstract BACKGROUND Between 1995 and 2002 the EORTC Brain Tumor Group conducted a prospective phase III study on adjuvant procarbazine, CCNU and vincristine (PCV) chemotherapy in anaplastic oligodendroglioma (AOD). A mature follow-up presented in 2012 showed survival benefit of the addition of PCV, in particular in 1p/19q co-deleted tumors and tumors with MGMT promoter methylation. We now present very long term follow-up. MATERIALS AND METHODS Patients were eligible if locally diagnosed with a newly diagnosed AOD. They were randomized between radiotherapy (RT, 33 x 1.8 Gy) and the same RT followed by 6 cycles PCV (RT/PCV). Primary endpoints were overall survival (OS) and progression free survival (PFS). 1p/19q status (FISH) was determined in 300 patient. Kaplan- Meier technique and Cox modeling were used for long term survival analysis. Primary analyses were adjusted for known prognostic factors. For other analyses no adjustment was performed. RESULTS With 368 patients included, a median follow-up of 18.4 years and 307 (83%) survival events, median and 20-year survival after RT/PCV versus RT alone were 42.3 mo and 16.8% vs 30.6 months and 10.1% (HR 0.78; 95% CI (0.63, 0.98), adjusted p=0.06). Eighty patients were 1p/19q codel of which 26 (33%) were still alive, in this subgroup median and 20-year survival after RT/PCV versus RT alone were 14 years and 37.1% versus 9.3 years and 13.6% (HR 0.60, 95% CI (0.35, 1.03), unadjusted p=0.06). Twenty year PFS in 1p/19q codel was 31.3% in RT/PCV treated patients and 10.8% in RT only treated patients (HR 0.49, 95% CI (0.29, 0.83), unadjusted p=0.007). In the 1p/19q codel subgroup age, WHO PS and necrosis at pathology were identified to be of independent prognostic value for OS. CONCLUSION This long term analysis confirms the earlier conclusions and provides data on long term survival in this patient group. In 1p/19q codel patients treated with RT/PCV, the 20-year PFS and OS rates are 31% and 37% respectively.


2012 ◽  
Vol 9 (5) ◽  
pp. 462-467 ◽  
Author(s):  
Timothy J. Hatlen ◽  
David B. Shurtleff ◽  
John D. Loeser ◽  
Jeffrey G. Ojemann ◽  
Anthony M. Avellino ◽  
...  

Object Programmable valves (PVs) for shunting CSF have increasingly replaced nonprogrammable valves (NPVs). There have been only a few longer-term studies (≥ 5 years) conducted that have compared the effectiveness of NPVs with that of PVs for children with hydrocephalus, and only 1 study has reported NPVs as being favorable over PVs. The objective of this retrospective study was to compare the long-term survival of these 2 types of shunt valves. Methods The authors collected data for all patients who underwent CSF shunt insertion or revision between January 1, 2000, and December 31, 2008. Patients underwent follow-up for a minimum of 2 years postoperatively. Statistical analyses were done using chi-square, Kaplan-Meier survival curve, and multivariate analyses. Results A total of 616 valves were implanted, of which 313 were PVs and 303 were NPVs. Of these, 253 were original shunt implantations and 363 were revisions. The proportion of 5-year survival for NPVs (45.8%) was significantly higher than that for PVs (19.8%) (p = 0.0005, log-rank). The NPVs that survived longer than 6 months also survived through the 5th year better than the PVs (p = 0.0001). Conclusions The authors' data suggest that NPVs survive longer than PVs in children, but there is a need for prospective, case-control studies to confirm these data.


2015 ◽  
Vol 210 (5) ◽  
pp. 904-910 ◽  
Author(s):  
Michiro Takahashi ◽  
Kiyoshi Hasegawa ◽  
Masaru Oba ◽  
Taku Aoki ◽  
Yoshihiro Sakamoto ◽  
...  

2020 ◽  
Vol 102-B (2) ◽  
pp. 198-204
Author(s):  
Rico Perlbach ◽  
Lars Palm ◽  
Maziar Mohaddes ◽  
Ingemar Ivarsson ◽  
Jörg Schilcher

Aims This single-centre observational study aimed to describe the results of extensive bone impaction grafting of the whole acetabular cavity in combination with an uncemented component in acetabular revisions performed in a standardized manner since 1993. Methods Between 1993 and 2013, 370 patients with a median age of 72 years (interquartile range (IQR) 63 to 79 years) underwent acetabular revision surgery. Of these, 229 were more than ten years following surgery and 137 were more than 15 years. All revisions were performed with extensive use of morcellized allograft firmly impacted into the entire acetabular cavity, followed by insertion of an uncemented component with supplementary screw fixation. All types of reoperation were captured using review of radiographs and medical charts, combined with data from the local surgical register and the Swedish Hip Arthroplasty Register. Results Among patients with possible follow-up of ten and 15 years, 152 and 72 patients remained alive without revision of the acetabular component. The number of deaths was 61 and 50, respectively. Of those who died, six patients in each group had a reoperation performed before death. The number of patients with a reoperation was 22 for those with ten-year follow-up and 21 for those with 15 years of follow-up. The Kaplan-Meier implant survival rate for aseptic loosening among all 370 patients in the cohort was 96.3% (95% confidence interval (CI) 94.1 to 98.5) after ten years and 92.8% (95% CI 89.2 to 96.6) after 15 years. Conclusion Extensive bone impaction grafting combined with uncemented revision components appears to be a reliable method with favourable long-term survival. This technique offers the advantage of bone stock restoration and disputes the long-standing perception that uncemented components require > 50% of host bone contact for successful implant survival. Cite this article: Bone Joint J 2020;102-B(2):198–204.


2020 ◽  
Vol 26 (5) ◽  
pp. 563-571 ◽  
Author(s):  
Victor M. Lu ◽  
Kyle P. O’Connor ◽  
Benjamin T. Himes ◽  
Desmond A. Brown ◽  
Cody L. Nesvick ◽  
...  

OBJECTIVEGlioblastoma (GBM) during infancy is rare, and the clinical outcomes of congenital GBM are not well understood. Correspondingly, the aim of this study was to present a long-term survivor case from the authors’ institution, and establish an integrated cohort of cases across the published literature to better understand the clinical course of this disease in this setting.METHODSThe authors report the outcomes of an institutional case of congenital GBM diagnosed within the first 3 months of life, and performed a comprehensive literature search for published cases from 2000 onward for an integrated survival analysis. All cases were integrated into 1 cohort, and Kaplan-Meier estimations, Fisher’s exact test, and logistic regression were used to interrogate the data.RESULTSThe integrated cohort of 40 congenital GBM cases consisted of 23 (58%) females and 17 (42%) males born at a median gestational age of 38 weeks (range 22–40 weeks). Estimates of overall survival (OS) at 1 month was 67%, at 1 year it was 59%, and at 10 years it was 45%, with statistically superior outcomes for subgroups in which patients survived to be treated by resection and chemotherapy. In the overall cohort, multivariable analysis confirmed resection (p < 0.01) and chemotherapy (p < 0.01) as independent predictors of superior OS. Gestational age > 38 weeks (p < 0.01), Apgar scores ≥ 7 at 5 minutes (p < 0.01), absence of prenatal hydrocephalus (p < 0.01), and vaginal delivery (p < 0.01) were associated with greater odds of surgical diagnosis versus autopsy diagnosis.CONCLUSIONSCongenital GBM can deviate from the expected poor prognosis of adult GBM in terms of OS. Both resection and chemotherapy confer statistically superior prognostic advantages in those patients who survive within the immediate postnatal period, and should be first-line considerations in the initial management of this rare disease.


Author(s):  
Ellen K Brinza ◽  
Lindsay Hagan ◽  
Arturo Evangelista ◽  
Eric M Isselbacher ◽  
Marek P Ehrlich ◽  
...  

Background: Young patients (pts) with acute aortic dissection (AAD) have distinct risk factors and presenting symptoms compared to older pts, but whether these differences extend past discharge is relatively unknown. Methods: Among pts presenting with AAD enrolled in the International Registry of Acute Aortic Dissection, pts <40 (N=280) were compared with pts ≥ 40 (N=3585). Chi-square analysis or Fischer’s Exact test were performed for categorical variables; age was compared using Student’s T-test. Kaplan-Meier curves were generated for freedom from adverse events rates 0-60 months following discharge. Mean follow-up was 28.6 months. Results: Significant differences in demographics and history were noted between pts <40 and the older cohort. Young pts more commonly had type A AAD (71.8%, 201/280, v. 64.6%, 2317/ 3585, p<0.016), while type B AAD was more typical in older pts (p<0.016). On imaging studies, pts <40 were less likely to present with IMH (7.3%, 246/3355, v. 2.3%, 6/266, p=0.002), but were more likely to have a patent false lumen (77.9%, 141/181, v. 62.1%, 1425/2295, p<0.001). Surgical management was more common in young pts, for both AAD types. In-hospital complications or mortality did not differ between groups. Kaplan-Meier analysis demonstrated better long-term survival in young pts compared to those ≥ 40 (p=0.029). Kaplan-Meier analyses of freedom from adverse events at 5 years illustrated no difference in aortic growth between groups, but significantly more late interventions in younger pts (p=0.006). Conclusions: Young pts show distinct differences in comparison to older pts, specifically regarding presentation, AAD type and management. Long-term survival and follow-up intervention rates are higher in young pts.


Stroke ◽  
2013 ◽  
Vol 44 (suppl_1) ◽  
Author(s):  
Matthew Thompson ◽  
Mohammad Issa ◽  
Marc Lazzaro ◽  
Osama Zaidat

Methods: We retrospectively reviewed clinical data on patients admitted at a single institution for possible stroke between 2004 and 2007, and selected subjects who underwent angiography of the neck. We classified subjects as having vertebral artery origin stenosis (VAOS) by ‘moderate’ to ‘severe’ (≥50%) occlusion. Age, sex, and race-matched control subjects were selected from our study population as having no evidence of VAOS on angiography. Long-term follow-up data was collected and death certificates were searched for comparison among cases and controls. A Kaplan-Meier curve was plotted based on time to event (stroke or death). Results: The proportion of subjects that were found to have VAOS was 58 per 358 cases, or 16.2%. Four subjects were excluded because of stenting, so a total of 54 cases and 54 matched controls were included for long-term follow-up analysis. In our study population, we calculated the relative risk of having a stroke or dying in patients with VAOS to be 6.0 times that of patients without VAOS ( p <0.02). The observed 5-year survival for patients with VAOS was 67% (36/54) compared to 89% (48/54) in control subjects ( p <0.01). Conclusions: Patients with vertebral artery origin stenosis are at a significantly higher risk of having a stroke or dying. Subsequent prospective, multicenter studies are needed to validate our results.


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