Evaluation of Exercise Capacity In Children with SCD by Six Minute Walk Test

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 2664-2664
Author(s):  
Caterina Minniti ◽  
Shoaib Alam ◽  
Gregory J. Kato ◽  
Mehdi Nouraie ◽  
Craig Sable ◽  
...  
Keyword(s):  
Multivariate Analysis ◽  
Exercise Capacity ◽  
Conflicts Of Interest ◽  
Strong Predictor ◽  
Univariate Analysis ◽  
History Of ◽  
Rate Of Decline ◽  
Changes Over Time ◽  
Minute Walk

Abstract Abstract 2664 Background: The six-minute walk (6MW) test is used in pediatrics in clinical practice and research to determine cardiopulmonary functional status. A change in 6MW may be affected by factors not strictly related to cardiopulmonary function, which may be different in different patient populations. In children and adolescents, age and height has been found to be a strong predictor of 6MW distance. We set out to study the effects of hematological and echocardiographic variables on 6MW distance in children with sickle cell disease (SCD) and its changes over time. Methods: We reviewed prospectively collected hematological, 6MW distance, and echocardiographic data from four hundred children with SCD (including 311 Hb SS or β0) and 69 controls (including 21 Hb AS) enrolled in PUSH (Pulmonary Hypertension and the Hypoxic Response in SCD). Subjects were evaluated at baseline and after 18–24 months, as per protocol. An un-encouraged 6MW was performed in children 5 years or older by trained personnel as per the guidelines of the American Thoracic Society. Subjects were studied at steady state, at least three weeks after any acute exacerbation of SCD. We used ANOVA for univariate analysis and stepwise linear regression for multivariate analysis. Results: Median (interquartile range) 6MW in severe SCD genotype (SS and S-β0) was 444 (399-508) and in controls was 495 meters (435-539) (P = 0.0002), while it was 461 meters (408-518) in milder SCD genotypes (P=0.2 for comparison with severe genotypes) (Table 1). In multivariate analysis, Hb, WBC and history of frequent pain episodes were significantly associated to distance of 6MW. Follow up 6MW obtained in 174 SCD subjects revealed a decline of 10% or more in distance in 22% of subjects with severe genotypes and 33% of other genotypes. The decline was more frequent in the subset of SS subjects with TRV>2.59 (40% vs 19%). CONCLUSION: Six minute walk distance is significantly shorter in children with SCD, even as young as 5 years of age, when compared to age and race appropriate controls, indicating early compromise of exercise capacity. SS and S-β-0 genotype subjects have more impairment of exercise capacity compared to milder genotypes. Predictors of 6MW distance are similar in SCD and non SCD subjects, which validates the use of this test in this patient population. Longitudinal changes in subjects with SCD are similar, with declines in about a quarter of the subjects. Patients with SS who have an elevated TRV have the highest rate of decline in 6MW. These results validate the use of 6MW as a tool for assessing exercise capacity in children with SCD. Disclosures: No relevant conflicts of interest to declare.

What Is the Appropriate Dose Attenuation System of RCHOP for Elderly DLBCL Patients? - A Single Institutional Analysis in 115 Cosecutive Patients From Japan -

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 3639-3639
Author(s):  
Akira Tanimura ◽  
Risen Hirai ◽  
Atsushi Sato ◽  
Miki Nakamura ◽  
Masataka Takeshita ◽  
...  
Keyword(s):  
Multivariate Analysis ◽  
Prognostic Factors ◽  
Elderly Patients ◽  
Conflicts Of Interest ◽  
Univariate Analysis ◽  
International Prognostic Index ◽  
Prognostic Index ◽  
B Cell Lymphoma ◽  
Age Related

Abstract Abstract 3639 Background: The combination therapy of RCHOP [rituximab (R), cyclophosphamide (CY), doxorubicin (DOX), vincristine (VCR), and prednisone (PSL)] is a standardized treatment for diffuse large B-cell lymphoma (DLBCL). However, its clinical outcome is worse in elderly patients because of comorbidities, age-related decrease in organ function, and impaired drug metabolism. If possible, the dose of RCHOP in elderly patients and patients with comorbidities should be adjusted appropriately. Since 2005, we have used a unified dose attenuation system for RCHOP according to the age and comorbidities of patients. This study retrospectively verified this system. Patients/Methods: We analyzed 115 consecutive DLBCL patients treated at our institute from September 2001, when rituximab was approved in Japan, to December 2010. From September 2001 to August 2005, 33 patients received dose adjustment of RCHOP according to the physician's discretion (PHY group). From September 2005, 82 patients received RCHOP according to the unified dose attenuation system (UNI group). In the UNI group, patients younger than 60 years received the standard RCHOP dose [R, 375 mg/m2; CY, 750 mg/m2; DOX, 50 mg/m2; VCR, 1.4 mg/m2 (max 2.0 mg/body); PSL, 100 mg/m2]. In patients older than 60 years, the doses of CY, DOX, VCR, PSL, and R were attenuated as shown in Table 1. In addition to age, the doses of CY, DOX, and VCR were adjusted according to organ functions (Table 2). The two groups were compared statistically. Results: The median age of patients was 70 years (range, 38–91), with 70.4% of patients classified as stage III or IV DLBCL, 40.4% with an international prognostic index (IPI) score of 0–2, and 70.2% with a ECOG performance status (PS) of 0 or 1. Low serum albumin levels (under normal range) were observed in 50.5% patients, and a high Charlson comorbidity index (CCI) score of >1 was found in 58.3%. The characteristics of the patients in the two groups were almost similar. The UNI system was completed in 94% of patients. The complete response (CR) rate was 63% in all patients (UNI group, 73%; PHY group, 39%; P = 0.0006). Univariate analysis revealed that better prognostic factors for CR were a low IPI score, better PS, and the UNI group. In the multivariate analysis, only the UNI group was a significantly better prognostic factor for CR. With a median follow-up of 26 months, the 5-year event-free survival (EFS) and overall survival (OS) were 39.3% and 68% in all patients, 43% and 72% in the UNI group, and 27% and 59% (5-year EFS; P = 0.0083, 5-year OS; P = 0.16) in the PHY group, respectively. Multivariate analysis showed that better prognostic factors for EFS were a low IPI score, a low CCI score, and the UNI group, and that for OS were low IPI and low CCI scores. In elderly patients aged >70 years (N = 59), the CR rates were 81% and 13% in the UNI and PHY groups, respectively (P = 0.0004), with OS in the UNI group being longer than that in the PHY group (72% vs. 59%; P = 0.02; Fig.1). In the UNI group, patient age did not affect the CR rate (<70, 71% vs. 70–79, 83% vs. >79, 79%; P = 0.56) or 5-year OS (<70, 76% vs. 70–79, 70% vs. >79, 66%; P = 0.58). The actual dose of CY, DOX, and VCR compared with the standard RCHOP dose was 64% and 26%, 63% and 16%, and 63% and 21% in the UNI and PHY groups, respectively. Disease progression during treatment, discontinuation of therapy, and death during treatment were observed in 10% and 15%, 5% and 24%, and 5% and 3% in the UNI and PHY groups, respectively. Nineteen patients (23%) from the UNI group died over a median follow-up of 15 months, while 15 patients (45%) of the PHY group died over a median follow-up period of 29 months. Lymphoma-related deaths were 12 (14%) in the UNI group and 8 (24%) in the PHY group. Five secondary primary malignancies (SPM) were observed (1 colon cancer and 1 breast cancer in the PHY group, and 1 lung cancer and 2 myelodysplastic syndrome in the UNI group). Four deaths were related to SPM. Conclusion: The unified dose attenuation system determined by the patients' age and comorbidities may achieve an effective dose level and better prognosis in elderly DLBCL patients. Disclosures: No relevant conflicts of interest to declare.

Abstract 19766: Prediction of Clinical Atrial Fibrillation induced during Typical Atrial Flutter Ablation

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Jorge Romero ◽  
Rodolfo Estrada ◽  
Anthony Holmes ◽  
David Goodman ◽  
Norman Roth ◽  
...  
Keyword(s):  
Atrial Fibrillation ◽  
Atrial Flutter ◽  
Multivariate Analyses ◽  
Strong Predictor ◽  
Univariate Analysis ◽  
Anticoagulation Management ◽  
Drug Induced ◽  
Typical Atrial Flutter ◽  
History Of

Background: Atrial fibrillation (AF) and isthmus dependent atrial flutter (AFL) are two separate entities that in many patients coexist. We sought to investigate whether AF inducibility (spontaneous or drug induced) during isthmus AFL ablation predicted the occurrence of AF at follow up after successful AFL ablation. Methods: Two hundred seventy three consecutive patients with isthmus dependent AFL undergoing ablation of AFL at our institution were enrolled in this study. 119 (43%) patients were excluded since they had evidence of AF prior to AFL ablation. Univariate and multivariate analyses were performed. Results: A total of 154 patients (male: 72%, age: 61 ±13) with AFL and without history of AF composed our patient population. All patients underwent successful AFL ablation. During ablation, AF was induced in 28 (18%) patients. After a mean follow up of 34 ± 23.5 months a total of 50 (32%) experienced AF. Univariate and multivariate analyses showed that only age and AF inducibility during AFL ablation were predictors of AF. Univariate analysis (age: p=0.038 and inducible AF p=0.032 and multivariate analysis (age: p=0.011 inducible AF: p=0.016) ) with and adjusted odds ratio of 3.3 [95% CI (1.250-8.676)] (Table 1). A total of 169 (62%) patients experienced AF before or after AFL ablation. Conclusion: AF inducibility in patients undergoing isthmus dependent AFL without history of AF is a strong predictor of AF recurrence. This has an important clinical relevance on anticoagulation management of these patients.

Predictors of restenosis after carotid artery stenting in 241 cases

2015 ◽  
Vol 8 (7) ◽  
pp. 677-679 ◽  
Author(s):  
Badih Daou ◽  
Nohra Chalouhi ◽  
Robert M Starke ◽  
Richard Dalyai ◽  
Adam Polifka ◽  
...  
Keyword(s):  
Risk Factors ◽  
Cardiovascular Disease ◽  
Multivariate Analysis ◽  
Carotid Artery ◽  
Cerebrovascular Accident ◽  
Carotid Artery Stenting ◽  
Univariate Analysis ◽  
Multivariate Logistic Regression ◽  
History Of

BackgroundVariable rates of restenosis after carotid artery stenting (CAS) have been reported, and few predictors have been suggested. Because CAS is being performed with increasing frequency, more data are needed to evaluate the rate and predictors of restenosis and possibly identify new risk factors for restenosis after CAS. The aim of this study was to analyze the rate and predictors of restenosis after CAS.Methods241 patients with carotid artery stenosis treated with stenting were analyzed retrospectively to identify patients who had restenosis after stenting. Univariate analysis and multivariate logistic regression were conducted to determine the predictors of restenosis.ResultsMean patient age was 67.5 years. 8.3% of patients who underwent CAS had carotid restenosis of ≥50% during follow-up. 3.7% of patients required retreatment. Mean duration from CAS to retreatment was 11 months. In multivariate analysis, the predictors of restenosis included history of cardiovascular disease (OR=8.88, p<0.001) and having a cerebrovascular accident (CVA) prior to stenting (OR=1.87, p=0.034). A higher percentage of preoperative carotid stenosis was associated with higher odds of restenosis in univariate analysis (p=0.04, OR stenosis ≥80%=5.7).ConclusionsOur results suggest that the rate of carotid restenosis after stenting is low. Patients with cardiovascular disease, patients who had a CVA prior to stenting, and patients with higher percentages of preoperative stenosis had higher odds of restenosis. Higher rates of restenosis should be kept in mind when opting for CAS in these patients.

scholarly journals Survival Advantage with Comparable Complete Chimerism Patterns in Reduced Toxicity Treosulfan-Based Vs Reduced Intensity Busulfan-Based Conditinioning Regimen in AML/MDS Patients Undergoing Allogeneic Haematopoietic Cell Transplantation

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 1914-1914
Author(s):  
Ioannis Batsis ◽  
Ioanna Sakellari ◽  
Apostolia Papalexandri ◽  
Despina Mallouri ◽  
Sofia Charalampidou ◽  
...  
Keyword(s):  
Multivariate Analysis ◽  
Conflicts Of Interest ◽  
Univariate Analysis ◽  
Unrelated Donor ◽  
Donor Chimerism ◽  
Significant Difference ◽  
Reduced Toxicity ◽  
Reduced Intensity ◽  
Grade Iii

Abstract Introduction: The optimal regimen for patients not fitting criteria for myeloablative conditioning has not yet been established. Treosulfan-based conditioning emerges as a potent antileukemic regimen with high efficacy and low toxicity in acute myeloid leukemia (AML) / myelodysplastic syndrome (MDS) patients. The current prospective study was designed to explore both safety and efficacy of FluTreo (fludarabine 150mg/m2, treosulfan 14 gr/m2/d ×3d) in medically infirm patients. Furthermore, we compared the outcome with a control similar population treated with FluBuATG (fludarabine 150mg/m2, busulfex 6.4mg/Kg, thymoglobulin-ATG 5-7.5mg/kg) reduced intensity conditioning. Method: A total of 20 patients, aged 54.5 (34-64) years, suffering from AML (14), MDS (2) and MDS related AML (4), after 3 (1-6) lines of treatment, received a FluTreo conditioning (plus ATG 5 mg/Kg in the setting of unrelated donor, MUD) from 10/2013-06/2015. They received peripheral grafts, CD34+ 7.0×106/kg from siblings (6 patients), 8/8 (9 patients) or 7/8 matched (5 patients) unrelated donors. The historic group consisted of 26 consecutive patients treated with FluBuATG during 2002-2012 for AML (20), MDS (5), MDS related AML (1). Chimerism evaluation in unfractionated bone marrow with STR (short tandem repeat) fragment analysis was performed regularly (on day + 14, + 30, +60, + 90) in both groups. Donor chimerism ≥99% was considered as complete donor chimerism (CDC). Results: The two groups were similar in terms of age, disease phase and risk group, lines of treatment or HCT-CI, except for more MUD in FluTreo. In the FluTreo group, neutrophil engraftment was achieved at day +10 (8-11) and platelet +12 (9-20), with 6 median days of neutropenia. Both groups experienced early engraftment: median time to CDC for both groups was + 30 days (range 12-69 in the Flutreo and 12-205 in the historic group). No significant difference was found between the two groups with respect to CDC on days +14 (30% vs 28%) or +30 (69% vs 57% respectively). Among patients receiving Flutreo 6 experienced CMV reactivation (2 infections), 7 EBV (1 PTLPD) and 1 tuberculosis. No grade III-IV organ associated toxicities were noted. Until last follow-up, 6 patients developed grade ≥II aGVHD and 9 cGVHD. Sixteen patients are alive in CR, while 4 died: 2 aGVHD grade III/IV, 1 cGVHD and 1 disease progression. With a median follow-up of 14 (2-130) for FluBuATG and 8.1 (2-20) months for FluTreo, 1-year disease-free survival was significantly higher in FluTreo (49% vs 88% respectively, p=0.009). Similarly, FluTreo showed a significant advantage in 1-year overall survival (OS) too (39% vs 77% respectively, p=0.006). In multivariate analysis, the regimen (FluBuATG vs FluTreo), age, disease phase, donor, lines of treatment, GVHD and HCT-CI were incorporated. In terms of OS, only the regimen (FluTreo) was recognized as a significantly favorable factor (p=0.029). FluTreo was also significant for superior DFS (p=0.016) along with less lines of treatment pre-transplant (p=0.026). Treatment related mortality (TRM), in univariate analysis, was significantly associated with MDS, time from diagnosis to transplant and previous lines of treatment, but none in multivariate analysis. Conclusions: This treosulfan-based reduced toxicity regimen seems safe and efficacious providing balanced immunosuppression that retains a graft-versus-leukemia effect with myeloablative activity and acceptable toxicity profile. Despite similar patterns of complete chimerisms compared to standard reduced intensity regimens, this regimen favors durable complete chimerism with low relapse rate. Prospective studies with longer follow-up are necessary to establish the advantages of this treosulfan-based regimen that render it a favorable alternative option in patients unfit for conventional regimens. Disclosures No relevant conflicts of interest to declare.

Comprehensive Genetic Screening of Chronic Myelomonocytic Leukemias (CMML)

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 3811-3811
Author(s):  
Raphael Itzykson ◽  
Olivier Kosmider ◽  
Aline Renneville ◽  
Margot Morabito ◽  
Céline Berthon ◽  
...  
Keyword(s):  
Multivariate Analysis ◽  
Conflicts Of Interest ◽  
Gene Dosage ◽  
Univariate Analysis ◽  
Continuous Variable ◽  
Prognostic Impact ◽  
Gene Dosage Effect ◽  
Disease Evolution ◽  
Mutational Status

Abstract Abstract 3811 Background: A large number of genes have been found mutated in CMML including 18 encoding signaling molecules (CBL, N/KRAS, JAK2, FLT3, KIT), epigenetic regulators (TET2, IDH1/2, DNMT3A, ASXL1, EZH2) transcription (RUNX1, NPM1) and splicing (SRSF2, SF3B1, U2AF1, ZRSR2) factors. We report the genotypic patterns, clinical correlates and prognostic impact of mutations in those 18 genes in a large cohort of CMML patients (pts). Methods: Bone marrow or peripheral CD14+ cells from 224 CMML pts from a non interventional study (n=186) or a phase II decitabine trial (n=38; Braun Blood 2011) were genotyped by mutation specific techniques and Sanger sequencing for up to 18 genes (depending on available material): TET2, IDH1, IDH2, DNMT3A, CBL, NRAS, KRAS, JAK2V617F, FLT3, KIT, NPM1, RUNX1, ASXL1, EZH2, SF3B1, SRSF2, U2AF1 and ZRSR2. The number of TET2 alleles with a functional Cystein Rich (CysR) domain (Delhommeau NEJM 2009) was predicted based on mutation type and zygosity, assuming that double mutations affect independent alleles. Overall (OS) and AML-free (AMLFS) survival were analyzed from the date of genotyping. Results: 224 CMML pts (152M/72F, median age 75y) were genotyped at diagnosis (37%) or after a median of 7.2 months of evolution (none had received hypomethylating agents [HMA] before genotyping); WHO diagnosis was CMML-1/2 in 78%/22%, 70% pts had normal karyotype, 22% had extramedullary disease (EMD); 13 pts had autoimmune manifestations (AIM). The most frequently mutated genes were TET2 (58%), SRSF2 (47%) and ASXL1 (38%). Mutations in RUNX1, CBL and NRAS were found in 14%, 11% and 10% of pts, respectively (resp). All other genes were mutated in <10% of pts. Only 5% pts lacked any mutation, and 70% had ≥2 mutated genes; TET2, IDH1 and IDH2 mutations, present in 64% of pts, were mutually exclusive. Mutations in splice and signaling genes were present in 63% and 35% of pts, with 2 mutated genes within each group in 3% and 2% pts, resp. ASXL1 mutations were less frequent in the presence of TET2 mutations (P<.0001). Significant mutual associations included ASXL1/RUNX1, ASXL1/NRAS, TET2/SRSF2, RUNX1/SRSF2 and U2AF1/IDH2. In multivariate analysis accounting for those interactions, TET2 status was the only independent predictor of hemoglobin values (median 10.2 vs 11.9 g/dL in wildtype [wt] vs mutated pts, P<.0001) with a gene dosage effect (P=.0003). Platelet counts were higher in JAK2V617F pts, and lower in pts with RUNX1, TET2 or SRSF2 mutations. WBC and monocyte counts were higher in pts with ASXL1 and NRAS mutations. EMD was associated to ASXL1, CBL, KRAS and JAK2 mutations. EMD, CMML-2 and abnormal karyotype were less frequent in TET2 mutated pts. All 13 pts with AIM had at least one mutated gene, with no specific genotype spectrum. With a median follow-up of 25.4 months, median OS and AMLFS were 32.2 and 28.0 months resp. In univariate analysis, OS was decreased in pts with IDH2 mutations (P=.04), and AMLFS was shorter in pts with NRAS (P=.04), RUNX1 (P=.03) and SRSF2 (P=.04) mutations. ASXL1 mutations markedly reduced OS (median 18.5 vs 35.7 months in wt pts) and AMLFS (median 12.5 vs 34.7 months, both P<.0001), with similar results in the 74 pts who received HMA during follow-up. In the 224 pts, there was no significant effect of overall TET2 status (wt vs mutated) on OS or AMLFS (both P=.09) but median OS was 29.3, 35.7 months and not reached in pts with 2 (57%), 1 (30%) and 0 (13%) TET2 alleles with a putatively functional CysR domain (P=.01). Similar differences were noted for AMLFS (P=.008). In multivariate analysis including peripheral blood counts, WHO classification, cytogenetics, disease evolution and therapy, ASXL1 was the only gene whose mutations independently predicted inferior OS (HR: 2.44, 95% CI: 1.36–4.37, P=.003)and AMLFS (HR: 2.54, 95% CI: 1.46–4.42, P=.001); SRSF2 mutations only predicted inferior AMLFS (HR: 2.05, 95% CI: 1.15–3.65, P=.02). The total number of mutated genes as a continuous variable, which was higher in ASXL1 mutated pts (mean 3.0 vs 1.8, P<.0001), was the only genetic variable to retain prognostic value when added to those models (OS and AMLFS both P<.0001). Conclusion: TET2, SRSF2 and ASXL1 are the most frequent mutated genes in CMML. The number and location of TET2 mutations may impact CMML presentation and outcome. The total number of mutated genes has the strongest prognostic relevance, but ASXL1 mutational status provides a robust surrogate prognostic marker for daily practice. Disclosures: No relevant conflicts of interest to declare.

scholarly journals PROGNOSTIC FACTORS OF RENAL TUMOR RECURRENCE AFTER RADICAL NEPHRECTOMY

2021 ◽  
Vol 108 (Supplement_3) ◽  
Author(s):  
G Martínez Izquierdo ◽  
A R Arnaiz Pérez ◽  
E Escolano Fernández ◽  
M Merayo Álvarez ◽  
B Carrasco Aguilera ◽  
...  
Keyword(s):  
Multivariate Analysis ◽  
Prognostic Factors ◽  
Radical Nephrectomy ◽  
Univariate Analysis ◽  
Malignant Neoplasms ◽  
Histopathological Analysis ◽  
Chi Square ◽  
Renal Sinus ◽  
Chi Square Test

Abstract INTRODUCTION Renal cell carcinoma (RCC) represents 3% of overall malignant neoplasms in adults. However, its aetiology has not been clearly established. Although surgery represents the cornerstone in treatment, recurrence postoperative rates are around 20-30%, what implies prognostic factors search must be mandatory in order to help to plan de follow-up and the different adjuvant therapy possibilities available in case they were necessary. MATERIAL AND METHODS A retrospective observational study was carried out in 110 patients who underwent radical nephrectomy between 2004 and 2018, with the aim of identifying possible prognostic factors of recurrence of RCC after these surgeries. Preoperative data (epidemiological, comorbidities and laboratory tests), surgical, pathological and variables related to follow-up were taken into account. A univariate and multivariate analysis were performed, using chi-square test and logistic regression, respectively. RESULTS The median follow-up time was 53.5 months (SD = 35.8), time in which 19 patients had a recurrence of RCC after radical nephrectomy (17.2%). Histopathological items such as the surgical piece size, the nodal and microvascular invasion, the renal sinus invasion and the presence of necrosis in the surgical piece were associated with RCC recurrence in the univariate analysis, while only the presence of necrosis in the surgical piece showed a significant result in the multivariate analysis (p = 0.004). CONCLUSIONS Histopathological analysis, highlighting the presence of necrosis in the histological sample, was proved to be the main risk factor of RCC recurrence.

scholarly journals Eculizumab discontinuation in atypical hemolytic uremic syndrome: TMA recurrence risk and renal outcomes

2021 ◽  
Author(s):  
Gema Ariceta ◽  
Fadi Fakhouri ◽  
Lisa Sartz ◽  
Benjamin Miller ◽  
Vasilis Nikolaou ◽  
...  
Keyword(s):  
Family History ◽  
Hemolytic Uremic Syndrome ◽  
Univariate Analysis ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Renal Response ◽  
Pathogenic Variants ◽  
Increased Risk ◽  
History Of ◽  
Uremic Syndrome

ABSTRACT Background Eculizumab modifies the course of disease in patients with atypical hemolytic uremic syndrome (aHUS), but data evaluating whether eculizumab discontinuation is safe are limited. Methods Patients enrolled in the Global aHUS Registry who received ≥1 month of eculizumab before discontinuing, demonstrated hematologic or renal response prior to discontinuation and had ≥6 months of follow-up were analyzed. The primary endpoint was the proportion of patients suffering thrombotic microangiopathy (TMA) recurrence after eculizumab discontinuation. Additional endpoints included: eGFR changes following eculizumab discontinuation to last available follow-up; number of TMA recurrences; time to TMA recurrence; proportion of patients restarting eculizumab; and changes in renal function. Results We analyzed 151 patients with clinically diagnosed aHUS who had evidence of hematologic or renal response to eculizumab, before discontinuing. Thirty-three (22%) experienced a TMA recurrence. Univariate analysis revealed that patients with an increased risk of TMA recurrence after discontinuing eculizumab were those with a history of extrarenal manifestations prior to initiating eculizumab, pathogenic variants, or a family history of aHUS. Multivariate analysis showed an increased risk of TMA recurrence in patients with pathogenic variants and a family history of aHUS. Twelve (8%) patients progressed to end-stage renal disease after eculizumab discontinuation; 7 (5%) patients eventually received a kidney transplant. Forty (27%) patients experienced an extrarenal manifestation of aHUS after eculizumab discontinuation. Conclusions Eculizumab discontinuation in patients with aHUS is not without risk, potentially leading to TMA recurrence and renal failure. A thorough assessment of risk factors prior to the decision to discontinue eculizumab is essential.

Distant metastases after diagnosis: Racial disparities in breast cancer outcomes.

2021 ◽  
Vol 39 (15_suppl) ◽  
pp. 1084-1084
Author(s):  
Julia Blanter ◽  
Ilana Ramer ◽  
Justina Ray ◽  
Emily J. Gallagher ◽  
Nina A. Bickell ◽  
...  
Keyword(s):  
Breast Cancer ◽  
Multivariate Analysis ◽  
Black Women ◽  
Racial Disparities ◽  
Late Stage ◽  
White Women ◽  
Univariate Analysis ◽  
Distant Metastases ◽  
Stage At Diagnosis

1084 Background: Black women diagnosed with breast cancer are more likely to have a poor prognosis, regardless of breast cancer subtype. Despite having a lower incidence rate of breast cancer when compared to white women, black women have the highest breast cancer death rate of all racial and ethnic groups, a characteristic often attributed to late stage at diagnosis. Distant metastases are considered the leading cause of death from breast cancer. We performed a follow up study of women with breast cancer in the Mount Sinai Health System (MSHS) to determine differences in distant metastases rates among black versus white women. Methods: Women were initially recruited as part of an NIH funded cross-sectional study from 2013-2020 to examine the link between insulin resistance (IR) and breast cancer prognosis. Women self-identified as black or white race. Data was collected via retrospective analysis of electronic medical records (EMR) between September 2020-January 2021. Distant metastases at diagnosis was defined as evidence of metastases in a secondary organ (not lymph node). Stage at diagnosis was recorded for all patients. Distant metastases after diagnosis was defined as evidence of metastases at any time after initiation of treatment. Univariate analysis was performed using Fisher’s exact test, multivariate analysis was performed by binary logistic regression, and results expressed as odds ratio (OR) and 95% confidence interval (CI). A p value <0.05 was considered statistically significant. Results: We identified 441 women enrolled in the IR study within the MSHS (340 white women, 101 black women). Median follow up time for all women was 2.95 years (median = 3.12 years for white and 2.51 years for black women (p=0.017)). Among these patients, 11 developed distant metastases after diagnosis: 4 (1.2%) white and 7 (6.9%) black (p=0.004). Multivariate analysis adjusting for age, race and stage at diagnosis revealed that black women were more likely to have distant metastasis (OR 5.8, CI 1.3-25.2), as were younger women (OR for age (years) 0.9, CI 0.9-1.0), and those with more advanced stage at diagnosis. Conclusions: Black women demonstrated a far higher percentage of distant metastases after diagnosis even when accounting for age and stage. These findings suggest that racial disparities still exist in the development of distant metastases, independent from a late-stage diagnosis. The source of existing disparities needs to be further understood and may be found in surveillance, treatment differences, or follow up.

Prognostic Factors for Developing Thrombosis in Polycytemia Vera: A Retrospective Analysis of 331 Patients with Long-Term Follow-up Highlights the Importance of White Blood Cells Levels

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 48-49
Author(s):  
Samantha Ferrari ◽  
Chiara Pagani ◽  
Mariella D'Adda ◽  
Nicola Bianchetti ◽  
Annamaria Pelizzari ◽  
...  
Keyword(s):  
Risk Factors ◽  
Multivariate Analysis ◽  
High Risk ◽  
Board Of Directors ◽  
Advisory Board ◽  
Advisory Committees ◽  
Risk Status ◽  
History Of ◽  
Wbc Count

Polycythemia Vera (PV) is a chronic myeloproliferative neoplasm characterized by erythrocytosis, constitutively active mutations in JAK2 and an increased susceptibility to thrombotic events (TEs). There is still controversy about the role of increased hematocrit and of other variables including elevated white blood cell count as risk factors for the occurrence of TEs. A better definition of the relative prognostic importance of hematologic parameters would help us to better tailor the therapeutic approach to PV patients (pts), which is currently mainly based on the use of acetilsalycilic acid (ASA), venesection and hydroxyurea . The aim of our study was to analyze if any clinical or laboratory variables were significantly associated to the occurrence of TEs both at PV diagnosis and during the course of the disease in a large series of PV pts uniformly followed at a single Center over a period of 29.5 years from January 1986 to June 2019. Clinical and laboratory data were obtained from the time of diagnosis until death, progression to acute leukemia or last follow-up. Hematocrit (Hct), hemoglobin (Hb), white blood cell (WBC) and platelet (PLT) levels were recorded for each patient at least every 6 months. Among a total of 331 pts, the median age was 65 years (range 30-92 years), and 56% were male. "High risk" features (age ≥ 60 years and/or history of prior thrombosis) were present in 221 pts (66.7%). The incidence of cardiovascular risk factors was: hypertension 64%, diabetes 15%, hyperlipidemia 28%, history of active or remote smoking 41%. Patients on ASA were 279 (84%), 19 (6%) were on oral anticoagulation, while 27 (8%) were on ASA+oral anticoagulant. At PV diagnosis 54 pts (16%) presented with thrombosis, arterial in 32 (59%) and venous in 22 (41%). A previous TE was recorded in 57 pts (17%): in 43 (75%) arterial, in 12 (22%) venous and in 2 (3%) mixed (arterial+venous). Previous thrombosis was the only variable significantly associated with the presence of a TE at PV diagnosis (P=0.02). After PV diagnosis, with a median follow-up of 81 months (range 1-374 months), 63 pts (19%) experienced a TE and 11 of them a further episode, for a total of 74 TEs. The incidence rate (pts/year) of TEs was 2.7%. Forty-two events were arterial (57%), 31 were venous (42%) and 1 (1%) was mixed. It was the first TE for 37 pts. Cerebrovascular accidents and deep-venous thrombosis were the most frequent arterial and venous TEs both at PV diagnosis and throughout the disease course, with a relative incidence of 50% and 32% respectively. The table compares the characteristics of patients who did or did not develop a TE after PV diagnosis. At univariate analysis, PV high risk status, a previous TE and hyperlipidemia at PV diagnosis were significantly associated with a subsequent TE. Among hematologic variables an elevated WBC count at the time of thrombosis, but not Hct or PLT levels, was highly significantly associated with the development of a TE. At multivariate analysis, WBC count ≥10.4 x 10^9/L and hyperlipidemia maintained their independent prognostic value, while high risk status and a previous TE lost their prognostic significance. Both at univariate and multivariate analysis, hyperlipidemia at diagnosis (P=0.009 and P=0.002) and high WBC count at thrombosis (P=0.001 and P=&lt;0.0001) predicted for arterial thromboses, while only a history of prior thrombosis (P=0.03) predicted for venous ones. In conclusion, our analysis confirms that elevated WBC count at the moment of the event more than increased hematocrit is associated to the development of thrombosis in PV pts. We also found that hyperlipidemia was an independent risk factor for arterial thrombosis, calling for an accurate management of increased lipid levels. Whether a reduction of the WBC count during the course of PV may reduce the frequency of TE remains to be demonstrated by prospective studies. Table Disclosures D'Adda: Novartis: Other: Advisory board; Incyte: Other: Advisory board; Pfizer: Other: Advisory board. Rossi:Daiichi Sankyo: Consultancy, Honoraria; Sanofi: Honoraria; Takeda: Honoraria, Membership on an entity's Board of Directors or advisory committees; Astellas: Membership on an entity's Board of Directors or advisory committees; Novartis: Other: Advisory board; Alexion: Membership on an entity's Board of Directors or advisory committees; Pfizer: Membership on an entity's Board of Directors or advisory committees; Amgen: Honoraria; Celgene: Membership on an entity's Board of Directors or advisory committees; Janssen: Membership on an entity's Board of Directors or advisory committees; Jazz: Membership on an entity's Board of Directors or advisory committees; Abbvie: Membership on an entity's Board of Directors or advisory committees.

Minor salivary gland biopsy: its importance in rheumatoid arthritis and secondary Sjögren's síndrome

2019 ◽  
pp. 15-18
Author(s):  
María Florencia Rodriguez ◽  
Cristian Troitiño ◽  
Felix Enrique Romanini ◽  
Anastasia Secco
Keyword(s):  
Rheumatoid Arthritis ◽  
Multivariate Analysis ◽  
Salivary Gland ◽  
Interstitial Lung Disease ◽  
Univariate Analysis ◽  
Minor Salivary Gland ◽  
Minor Salivary Gland Biopsy ◽  
Extraglandular Manifestations ◽  
Salivary Gland Biopsy

Secondary Sjögren’s Syndrome (SSs) is common in patients with Rheumatoid Arthritis (RA). Objectives: to determine if lymphocytic sialoadenifitis (FLS) is associated with clinical and serological differences in a group of patients with RA and SSs. Methods: patients with a diagnosis of RA and SSs were included, which of them presented FLS in the salivary gland biopsy, clinical and serological characteristics were compared. Results: 88 patients were included, 92% women, mean age of 53 years (SD ± 11.3) and 12.5 years of evolution of RA (RIC 6-7). 63.6% had SLF versus 36.4% who did not. In the univariate analysis, a statistically significant association was found between FLS + and the variables: parotidomegaly, interstitial lung disease, hypergammaglobulinemia, hypocomplementemia, rheumatoid factor, positive ANF, and extra-articular and/ or extraglandular manifestations. In the multivariate analysis, the variables independently associated with the presence of FLS were: extra-articular and/or extraglandular manifestations (OR 5.67, 95% CI 1.6-20), positive ANF (OR 11.7, 95% CI 1.6-83) and hypergammaglobulinemia (OR 21, 95% CI 2.46-179). Conclusion: patients with RA and SSs with FLS have a higher frequency of extra-articular and extraglandular manifestations and serological differences, which would imply a different clinical follow-up.

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