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Objective To assess the prevalence and pattern of myocardial fibrosis as detected by delayed enhanced magnetic resonance imaging (DE‐MRI) in patients with systemic sclerosis (SSc), and to evaluate a possible association between myocardial fibrosis and cardiac arrhythmias. Methods Forty‐one patients with SSc underwent 24‐hour Holter monitoring, Doppler echocardiography, and DE‐MRI following gadolinium administration. Results Technically acceptable DE‐MRIs were obtained in 36 patients with SSc. Enhancement on DE‐MRI, consistent with myocardial fibrosis, was observed in 24 of these patients (66%), and it was invariably midwall with a linear pattern, mostly involving basal and midcavity segments of the left ventricle. The volume of enhancement (total volume percentage index [TVPI]) did not differ between patients with diffuse SSc and those with limited SSc (mean ± SD 1.46 ± 1.73% versus 1.44 ± 1.77%; P = 0.98). Patients with a long duration (≥15 years) of Raynaud's phenomenon had a greater number of enhancing segments (mean ± SD 6.55 ± 4.93 versus 2.96 ± 3.46; P = 0.017) and a greater TVPI (mean ± SD 2.44 ± 1.97% versus 1.02 ± 1.43%; P = 0.02) than those with a duration of Raynaud's phenomenon <15 years. Nineteen patients with SSc (53%) had abnormal Holter study results. Compared with patients with normal Holter study results, those with abnormal results had a greater number of enhancing segments (mean ± SD 5.4 ± 4.8 versus 2.5 ± 2.9; P < 0.05) and a greater TVPI (mean ± SD 2.1 ± 1.9% versus 0.8 ± 1.2%; P < 0.05). Conclusion DE‐MRI can identify myocardial fibrosis in a significant percentage of patients with SSc and may be a useful noninvasive tool for determining cardiac involvement.

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The 8p11 myeloproliferative syndrome (EMS) is a rare hematologic malignancy characterized by myeloid hyperplasia, eosinophilia, and precursor lymphoblastic lymphoma, associated with balanced translocations involving chromosome 8p11, most commonly t(8;13)(p11;q12). Approximately 75% of EMS patients present with or develop precursor T-cell lymphoblastic lymphoma, and most subsequently develop acute myeloid leukemia. Here we describe the morphologic and immunophenotypic features of 6 cases of t(8;13)-positive bilineal lymphoma of mixed T-cell and myeloid lineage, 5 in lymph nodes and 1 in breast. The patients, 3 males and 3 females, ranged in age from 6 to 19 years. Histologically, each tumor was composed of 2 distinct cellular components: small to medium-sized T cells with scant cytoplasm that resembled lymphoblasts, and larger immature-appearing cells with more abundant eosinophilic cytoplasm that resembled myeloblasts, a subset of which expressed myeloid antigens. In all cases, the latter component tended to surround residual lymphoid follicles and/or blood vessels. Numerous eosinophils and prominent high endothelial venules were present in all of the lymph node specimens. Interestingly, cells of both components expressed CD3 on immunohistochemical stains. In conclusion, EMS associated with t(8;13) should be suspected in patients with a bilineal tumor that involves lymph nodes or other extramedullary sites. We believe that these bilineal neoplasms of mixed T-cell and myeloid lineages, which present as lymphoma, are analogous to bilineal leukemias. They likely arise from an early hematopoietic cell with potential to differentiate along T-cell and myeloid pathways.

ObjectiveTo evaluate the role of concurrent systemic therapy to postoperative radiation therapy (RT) for locally advanced cutaneous head and neck squamous cell carcinoma (LA-cHNSCC).Materials and methodsA retrospective study of 32 patients with LA-cHNSCC receiving postoperative RT with and without systemic therapy was conducted. Patients with LA-cHNSCC after surgical resection with one or more high risk features were evaluated. Local regional control (LRC), distant control (DC), and acute and late toxicities were evaluated with Fisher exact tests. Progression-free survival (PFS) and overall survival (OS) were evaluated utilizing Kaplan Meier and log-rank analyses. Univariate Cox proportional hazard analyses were used to examine patient, disease, and treatment-related factors with OS and PFS.ResultsWhile comparing patients receiving RT with systemic therapy (n = 14) vs RT alone (n = 18), LRC was 92.9% vs 72.2% (p = 0.20), DC 92.9% vs 94.4% (p = 1.0), median PFS 17.7 months vs 34.4 months (p = 0.48), and median OS 20.9 months vs 34.4 months (p = 0.03), respectively. On univariate analyses, use of concurrent systemic therapy was associated with an increased risk of death with an HR of 3.5 [95% confidence interval (CI): 1.04 - 11.6] (p = 0.04), while patients treated for recurrent disease who had previously treated superficial primaries had improved OS with an HR of 0.10 [95% CI: 0.01-0.80] (p = 0.03). There were no significant differences in acute or chronic toxicities between groups.ConclusionsPatients receiving postoperative RT alone for LA-cHNSCC had better OS than patients receiving concurrent systemic therapy. There were no differences in any other endpoints evaluated.

PURPOSE: During radiotherapy (RT), patient symptoms are evaluated and managed weekly during physician on-treatment visits (OTVs). The Edmonton Symptom Assessment Scale (ESAS) is a 9-symptom validated self-assessment tool for reporting common symptoms in patients with cancer. We hypothesized that implementation and physician review of ESAS during weekly OTVs may result in betterment of symptom severity during RT for certain modifiable domains. METHODS: As an institutional quality improvement project, patients were partitioned into 2 groups: (1) 85 patients completing weekly ESAS (preintervention) but blinded to their providers who gave routine symptom management and (2) 170 completing weekly ESAS (postintervention group) reviewed by providers during weekly OTVs with possible intervention. To determine the independent association with symptom severity of the intervention, multivariate logistic regression was performed. At study conclusion, provider assessments of ESAS utility were also collected. RESULTS: Compared with the preintervention group, stable or improved symptom severity was seen in the postintervention group for pain (70.7% v 85.6%; P = .005) and anxiety (79.3% v 92.9%; P = .002). The postintervention group had decreased association (on multivariate analysis) with worsening severity of pain (OR, 0.13; P < .001), nausea (OR, 0.25; P = .023), loss of appetite (OR, 0.30; P = .024), and anxiety (OR, 0.19; P = .005). Most physicians (87.5%) and nurses (75%) found ESAS review useful in symptom management. CONCLUSION: Incorporation of ESAS for OTVs was associated with stable or improved symptom severity where therapeutic intervention is more readily available, such as counseling, pain medication, anti-emetics, appetite stimulants, and anti-anxiolytics. The incorporation of validated patient-reported symptom-scoring tools may improve provider management.

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