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We included 594 patients, 297 with GCA (73% female), mean age at diagnosis 75 years. The rate of statin exposure at index date was 18.1% for GCA patients versus 33.3% for controls (p < 0.001). Patients using statins were less likely to develop GCA compared with patients not using statins (OR 0.31, 95% CI 0.15-0.6, p < 0.001), even after adjustment for cardiovascular risk factors. Among patients with GCA, the presenting clinical features and acute-phase reactants were similar in patients receiving statins compared to those not on statin therapy. These 2 groups were also similar with regard to relapse rate, prednisone tapering, and overall survival.
This is a retrospective cohort study.
To assess the safety and efficacy of abiraterone acetate (AA) in patients with metastatic castration-resistant prostate cancer (mCRPC) treated in a compassionate named patient programme (NPP).
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The purpose of this study was to evaluate the activity of Selective Inhibitors of Nuclear Export (SINE) compounds that inhibit the function of the nuclear export protein Exportin 1 (XPO1/CRM1) against canine tumor cell lines and perform a Phase I clinical trial of KPT-335 in dogs with spontaneous cancer to provide a preliminary assessment of biologic activity and tolerability.
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Contrary to prior reports, our data suggests that outcomes of primary adrenal DLBCL are encouraging using a regimen of R-CHOP, and that achieving CR after R-CHOP is predictive of survival. Likewise, our modified staging system may have prognostic value.
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We report a case of the extremely rare condition Epstein-Barr virus (EBV)-positive T-cell lymphoproliferative disease (LPD) which occurred after umbilical cord blood transplantation. A 25-year-old Japanese man underwent cord blood transplantation from a male human leukocyte antigen 4/6-matched donor due to acute myeloid leukemia with trisomy 8. Bone marrow examination on day 30 showed chimerism with at least 90% donor cells and complete hematological response. Chronic symptoms of graft-versus-host disease appeared only on the skin and were successfully treated with cyclosporine alone. Three years later, however, the patient experienced repeated cold-like symptoms and was hospitalized with liver dysfunction. A high fever developed and was followed by significant edema of the right side of the face. The EBV DNA copy number in whole peripheral blood was 2×10(4)/mL. Liver biopsy showed invasion of EBV-infected CD8-positive T cells. Southern blotting analysis of the whole peripheral blood showed that the T-cell receptor Cβ1 rearrangement was positive. On the basis of these results, EBV-positive T-cell LPD was diagnosed and treated with prednisolone, cyclosporine, and etoposide, followed by cyclophosphamide, doxorubicin, vincristine, and prednisone. However, the patient died of cardiac function failure, pneumonia, and pulmonary hemorrhage, all of unidentified cause. Most cases of EBV-related LPD after hematopoietic stem cell transplantation consist of EBV-positive B-cell LPD, and, to our knowledge, de novo EBV-positive T-cell LPD subsequent to transplantation has not been previously reported.
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Full data were available for 152 patients, of whom 28 had never been vaccinated against pneumococcus. Median levels were significantly higher in those who had been vaccinated. Unvaccinated patients and those taking oral prednisone were more likely to have had pneumonia in the previous 10 years. The RR for developing pneumonia among non-vaccinated patients was 9.7 (p=0.005) and among steroid-treated patients was 6.5 (p=0.001), after adjusting for age, gender, disease duration and comorbidity. No significant correlation was found between pneumococcal antibody levels and time since vaccination.
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Viral hepatitis is common worldwide and in the United States. Inflammatory bowel disease (IBD) patients with chronic hepatitis B virus (HBV) with active disease (elevated alanine aminotransferase level and viral replication) should receive HBV treatment. HBV reactivation is associated with significant morbidity and mortality in patients receiving immunosuppression. IBD patients may require long-term immunosuppression, and therefore should be considered candidates for vaccination against new HBV infection as well as prophylaxis against HBV reactivation prior to immunosuppressive therapy. Tumor necrosis factor alpha antagonists and immunomodulators appear compatible with use in IBD patients with HCV, although prednisone may increase viral replication. HCV treatment with peg-interferon and ribavirin may exacerbate gastrointestinal symptoms, and therefore the decision to treat HCV needs to be individualized. Management of IBD patients with viral hepatitis is addressed in this review.
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Orbital myositis is a rare extra-intestinal manifestation of inflammatory bowel disease. Seventeen cases of Crohn's disease associated orbital myositis and 3 cases of ulcerative colitis associated orbital myositis have been reported in the published literature since 1970. We report the use of adalimumab (Abbott, Canada, Inc.) for orbital myositis in a patient with Crohn's disease who discontinued infliximab (Janssen, Canada, Inc.) and review of the published literature.
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Hemangiomas are the most common benign tumors of the liver. They are generally asymptomatic, but giant hemangiomas can lead to abdominal discomfort, bleeding, or obstructive symptoms. Kasabach-Merritt syndrome is a rare but life-threatening complication of hemangioma, characterized by consumptive coagulopathy with large vascular tumors. More than 80% of Kasabach-Merritt syndrome cases occur within the first year of life. However, there are few reports of Kasabach-Merritt syndrome with giant hepatic hemangioma in adults and, as far as we know, no reports of Kasabach-Merritt syndrome with hepatic hemangioma treated with first line medical treatment only. The most important treatment for this syndrome is removal of the large vascular tumor. However, surgical treatment entails risk of bleeding, and the patient's condition can mitigate against surgery. We herein present a case of unresectable giant hepatic hemangioma with disseminated intravascular coagulopathy. The patient was a 60-year-old woman who complained of hematochezia, ecchymosis, and abdominal distension. She refused all surgical management and was therefore treated with systemic glucocorticoids and beta-blockers. After two weeks of steroid therapy, she responded partially to the treatment. Her laboratory findings and hematochezia improved. She was discharged on hospital day 33 and observed without signs of bleeding for three months.
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Primary bladder lymphoma, a rare form of non-Hodgkin's lymphoma that is confined to the urinary bladder, is usually of B-cell origin. This report describes an extremely rare case of primary T-cell lymphoma of the urinary bladder. A 45-year-old man presented with haematuria, dysuria and loin pain. Ultrasonography and computed tomography showed a thickened left bladder wall and left hydroureteronephrosis. A diagnosis of primary T-cell lymphoma of the urinary bladder was made on the basis of clinical, radiological and histological findings. The patient, following transurethral resection, was treated with four cycles of CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) chemotherapy with a good response and remains in clinical remission 12 months after treatment.
Giant cell arteritis (GCA) is the most common systemic vasculitis affecting people over 50 years. This disease is a diagnostic challenge with a range of clinical symptoms and findings due to different affected vessels. Because of this, the initial diagnosis can be tricky, and some of the patients present at first time with a real unusual initial manifestation. One of these can be tongue necrosis, which is according to the literature in accordance with scalp necrosis, the rarest initial manifestation of GCA We describe a patient who presented with tongue necrosis as initial symptom of GCA. The belated diagnose resulted in subtotal necrosis of the mobile part of the tongue.
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The total number of patients included was 121. Median age was 57 years. After central review, interim PET (n = 111) was negative in 76% of patients, and final PET (n = 106) was negative in 78%. With a median follow-up of 23 months, 2-year progression-free survival rates were 86% for interim PET-negative versus 61% for interim PET-positive patients (P = .0046) and 87% for final PET-negative versus 51% for final PET-positive patients (P < .001), respectively. Two-year overall survival also significantly differed according to final PET results: 100% versus 88% (P = .0128).
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The optimal treatment strategy for primary mediastinal large B-cell lymphoma (PMBL) remains unknown. We retrospectively analyzed 345 patients with newly diagnosed PMBL to identify prognostic factors and optimal treatments. Focusing on patients treated with cyclophosphamide, doxorubicin, vincristine, prednisolone, and rituximab (R-CHOP) (N=187), a higher International Prognostic Index (IPI) and the presence of pleural or pericardial effusion were identified as adverse prognostic factors for overall survival (OS) in patients treated with R-CHOP without consolidative radiation therapy (RT) [IPI: hazard ratio (HR), 4.23; 95% confidence interval (CI), 1.48-12.13; P=0.007; effusion: HR, 4.93; 95% CI, 1.37-17.69; P=0.015]. Combined with IPI and the presence of pleural or pericardial effusion for the stratification of patients treated with R-CHOP without RT, those with lower IPI and the absence of effusion comprised approximately onehalf of these patients and could be identified as curable [OS and progression free survival (PFS) at 4 years, 95% and 87%, respectively)] Taken together, our simple indicators of IPI and the presence of effusion could stratify patients with PMBL and thereby facilitate treatment selection.
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Diffuse large B-cell lymphoma can be classified by gene expression profiling into germinal center and activated B-cell subtypes with different prognoses after rituximab-CHOP. The importance of previously recognized prognostic markers, such as Bcl-2 protein expression and BCL2 gene abnormalities, has been questioned in the new therapeutic era. We analyzed Bcl-2 protein expression, and BCL2 and MYC gene abnormalities by interphase fluorescence in situ hybridization in 327 patients with de novo disease treated with rituximab-CHOP. Isolated BCL2 and MYC rearrangements were not predictive of outcome in our patients as a whole, but only in those with the germinal center subtype of lymphoma. The prognostic relevance of isolated MYC rearrangements was weaker than that of BCL2 isolated translocations, but was probably limited by the rarity of the rearrangements. Seven of eight patients with double hit lymphoma had the germinal center subtype with poor outcome. The germinal center subtype patients with isolated BCL2 translocations had significantly worse outcome than the patients without BCL2 rearrangements (P=0.0002), and their outcome was similar to that of patients with the activated B-cell subtype (P=0.30), but not as bad as the outcome of patients with double hit lymphoma (P<0.0001). Bcl-2 protein overexpression was associated with inferior outcome in patients with germinal center subtype lymphoma, but multivariate analysis showed that this was dependent on BCL2 translocations. The gene expression profiling of patients with BCL2 rearrangements was unique, showing activation of pathways that were silent in the negative counterpart. BCL2 translocated germinal center subtype patients have worse prognosis after rituximab-CHOP, irrespective of MYC status, but the presence of combined gene breaks significantly overcomes the prognostic relevance of isolated lesions.
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Complex regional pain syndrome type 1 is a painful disorder that may complicate a traumatic lesion especially in extremities. It is infradiagnosed and potentially very disabling. Its diagnosis consists of a few clinical criteria. It does not exist a complementary diagnostic study which can be considered as gold standard. Its treatment must be multidisciplinary (e.g. medicines, physical therapy, psychological therapy and, in selected cases, invasive procedures). There have been communicated some favorable results using multiple pharmacological treatments in which corticosteroid therapy was among the ones that have reached highest levels of evidence in favor of its utility. Here we present the case of a forty-seven male patient who presented severe pain in his right lower extremity with neuropathic characteristics and a long time of evolution. He had had multiple previous consultations and he had carried out many other treatments. It has been performed a 99Tc three-phase bone scintigraphy that was compatible with the proposed diagnosis. He was treated with methyl prednisone 60 mg q24h with progressive lowering doses during fifteen days with a significant improvement of signs and symptoms. Finally he received hospital discharge for ambulatory rehabilitation treatment.
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7 September 2009 on ClinicalTrials.gov: NCT00973154.
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TCZ treatment was associated with rapid and maintained clinical and laboratory improvement in patients with AOSD refractory to standard treatment. However, joint manifestations seem to be more refractory to treatment compared with systemic manifestations.
This case showed that physicians should be aware of the potential side effect of metformin although it is infrequent.
Acne fulminans is a rare condition and the most severe form of acne. It involves the sudden onset of febrile and multisystemic symptoms, with poor response to ordinary therapy in patients who previously had mild to moderate acne. It is characterized by hemorrhagic ulcerative crusting lesions on the face, chest and upper back. The authors report a case of acne fulminans that was successfully treated with oral prednisone and dapsone.
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Adult idiopathic isolated ACTH deficiency (AIIAD) is an underestimated disorder which is frequently misdiagnosed. We presented 3 new Chinese AIIAD cases, summarized their clinical characteristics and analyzed the available literature.
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This prospective study included 19 patients (38 eyes) diagnosed with acute uveitis associated with VKH disease.
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目的：通过分析1例累及气管及气管周围软组织IgG(4)相关性疾病的临床资料并复习文献，提高对该病的认识与诊治水平。方法：分析1例累及气管及气管周围软组织的IgG(4)相关性疾病患者的临床表现、实验室检查及影像、组织病理、治疗及预后，并进行相关文献复习。结果：患者女，18岁，因咳嗽、呼吸困难2月余，发现颈部肿物1月余入院。颈部CT显示，颈段气管不规则狭窄，气管周围软组织广泛弥漫增厚。气管旁肿物活检病理显示，增生的致密胶原纤维组织，见较多淋巴细胞、浆细胞浸润，免疫组化：IgG(4)阳性浆细胞数>50个/HP，IgG(4)阳性细胞数/IgG阳性细胞数>40%。血清IgG(4)水平2 930 mg/L，诊断IgG(4)相关性疾病。静脉点滴甲泼尼龙80 mg/d，3 d后序贯口服泼尼松40 mg/d，症状缓解，1个月后复查颈部CT显示，颈段气管狭窄明显改善，气管周围软组织明显缩小。检索英文文献，共20例累及气管及气管周围软组织的IgG(4)相关性疾病，仅单纯累及气管壁及气管旁软组织者1例，同时伴有气管外受累者19例。12例检测血清IgG(4)，11例升高(6 150～34 050 mg/L)。18例患者接受口服糖皮质激素治疗，部分联合免疫抑制剂或利妥昔单抗治疗，预后良好。结论：累及气管及气管周围软组织的IgG(4)相关性疾病临床少见，应积极完善组织病理学检查，糖皮质激素治疗有效，预后相对良好。.
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Intetumumab is a fully human mAb with antiangiogenic, antitumor properties which has shown potential therapeutic effect in castration-resistant prostate cancer (CRPC) patients.
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The phase III trial GEM05MENOS65 randomized 390 patients 65 years old or younger with newly diagnosed symptomatic multiple myeloma (MM) to receive induction with thalidomide/dexamethasone, bortezomib/thalidomide/dexamethasone and Vincristine, BCNU, melphalan, cyclophosphamide, prednisone/vincristine, BCNU, doxorubicin, dexamethasone bortezomib (VBMCP/VBAD/B) followed by autologous stem cell transplantation (ASCT) with MEL-200. After ASCT, a second randomization was performed to compare thalidomide/bortezomib (TV), thalidomide (T) and alfa-2b interferon (alfa2-IFN). Maintenance treatment consisted of TV (thalidomide 100 mg daily plus one cycle of intravenous bortezomib at 1.3 mg/m(2) on days 1, 4, 8 and 11 every 3 months) versus T (100 mg daily) versus alfa2-IFN (3 MU three times per week) for up to 3 years. A total of 271 patients were randomized (TV: 91; T: 88; alfa2-IFN: 92). The complete response (CR) rate with maintenance was improved by 21% with TV, 11% with T and 17% with alfa2-IFN (P, not significant). After a median follow-up of 58.6 months, the progression-free survival (PFS) was significantly longer with TV compared with T and alfa2-IFN (50.6 vs 40.3 vs 32.5 months, P=0.03). Overall survival was not significantly different among the three arms. Grade 2-3 peripheral neuropathy was observed in 48.8%, 34.4% and 1% of patients treated with TV, T and alfa2-IFN, respectively. In conclusion, bortezomib and thalidomide maintenance resulted in a significantly longer PFS when compared with thalidomide or alfa2-IFN. (no. EUDRA 2005-001110-41).Leukemia advance online publication, 14 February 2017; doi:10.1038/leu.2017.35.
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Myasthenia gravis (MG) is an immune-mediated disorder with a variable response to treatment. In this study, patients with refractory MG who were treated with rituximab were identified. A review of patients referred to the Yale Neuromuscular Clinic was performed. Patients with refractory MG who were treated with rituximab were reviewed for response to treatment. Patients who had muscle-specific kinase (MuSK(+)) or acetylcholine receptor (AChR(+)) antibodies were included. Six patients were identified who met the criteria described. All patients tolerated rituximab without side effects and had a reduced need for immunosuppressants and/or improvement in clinical function. Patients with refractory MG appeared to respond to rituximab in this small, retrospective study. This result suggests that a larger, prospective trial is indicated.