Modified IDARAM chemotherapy regimen for primary central nervous system lymphoma: experience of three cases

The use of radiotherapy (RT) with chemotherapy has improved disease free survival and control in primary central nervous system lymphoma (PCNSL). We have encountered three patients with histologically documented central nervous system lymphoma. In all patients pathological diagnosis was B-cell lymphoma. We modified IDARAM regimen to R-IDARAM to enhance and optimize chemotherapeutic components for the treatment of PCNSL. We made three changes: (i) we added rituximab 375 mg/m(2) day 1; (ii) increased dose of MTX from 2 to 3 g/m(2); and (iii) administered two additional courses of R-IDARAM after cranial RT. Following complete staging after course 2, radiotherapy was applied at a dosage of 3600-4140 cGy in conventional schedule (180 or 200 cGy per day) to whole brain (with 3600 cGy to eyes in one case because of eye involvement) and then 2 additional courses of R-IDARAM (totally four courses) chemotherapy regimen were applied. Complete remission (CR) was achieved after first two cycles of R-IDARAM in patient 1 and 3 and after four cycles in patient 2. Currently, three patients have been alive for 29, 10, 15 months respectively. Currently there is no standard treatment modality for PCNSL. Increased dosage of MTX, adding rituximab and consolidation of the IDARAM to R-IDARAM regimen may improve disease control and outcome in PCNSL patients.     

Novel Βeta (β)-Thalassemia Mutation in Turkish Children

Beta (β)-thalassemia is the most frequently observed hereditary blood disorder in the world. It is characterized by deficiency of hemoglobin β-globin gene and is also a profoundly heterogeneous both at the molecular and clinical level. In the case of β-thalassemia, there is reduced (β⁺ type) or absent (β^o type) synthesis of the beta chains of hemoglobin. β-Thalassemia clinically occurs in three main forms: major, intermedia and minor according to requirement of transfusion. The objective of this study was to evaluate β-thalassemia mutations in 89 patients ranging from 2 months to 16 years of age, who enrolled to Medical School Research and Training Hospital, Gaziantep University. The direct DNA sequence analysis was performed for mutation scanning of β-globin gene. 89 children with β-Thalassemia including all types were analyzed, 16 different β-thalassemia mutations were detected. We have also identified a novel mutation (HBB.c.-80delT, rs397509430) in the promoter region (−30 TATA box) of β-globin gene, and clinical data of patient having novel mutation was given. The β-Thalassemia mutations were determined as β-Thalassemia major type in 42 patients (47.19 %), β-Thalassemia intermedia in 4 (4.49 %), β-Thalassemia minor in 43, (48.31 %) patients. The most frequent mutation was IVS I-110 G>A, followed by IVS I-1 G>A, IVS I-6 T>C, IVS II-1 G>A, respectively.     

Percutaneous transluminal angioplasty for total coronary occlusion: The effect of restenosis on left ventricular function

The study was undertaken to evaluate the effect of restenosis on global and regional left ventricular function after percutaneous transluminal coronary angioplasty (PTCA) for total coronary occlusion. Thirty-one consecutive patients with total coronary occlusion treated successfully with PTCA and had follow-up angiography at 3–6 months formed the study group. Nineteen patients had restenosis (16 males, mean age 48±10 years) and 12 had no restenosis (11 males, mean age 53±10 years). In the LAD group there were increases in anterobasal (35.9±8.5% vs 43.1±5.7%, p=0.05) and apical (24.1±6.8% vs 31.7±2.9%, p=0.03) segment motion scores in patients without restenosis after PTCA. Global ejection fractions (63.1±14.5% vs 68.9±12.4%, p=0.09) and anterolateral (28.7±11.3% vs 39.7±10.2%, p=0.09) segment scores increased, but did not reach statistical significance. In the LAD restenosis group anterobasal (41.5±14.3% vs 34.3±12.6%, p=0.001), apical (21.1±15.0% vs 17.8±10.9%, p=0.05) and anterolateral (32.7±19.6% vs 26.6±13.8%, p=0.03) segment motion scores decreased but the decrease in the global ejection fraction (60.3±18.5% vs 58.6±17.4, p=0.38) was not significant. In the RCA+LCX group there was a significant increases in global ejection fraction (69.0±7.5% vs 74.2±7.6%, p=0.03) and posterobasal (23.8±7.8 vs 34.4±8.0, p=0.04) segment motion scores in patients without restenosis. The wall motion scores were unchanged in patients with restenosis in the RCA+LCX group. It was concluded that restenosis after a successful PTCA for total coronary occlusion may deteriorate segmental wall motion and treatment modalities with increased patency rates should be used for total coronary occlusions.     

Relationship Between Antihypertensive Medications and Cognitive Impairment: Part I. Review of Human Studies and Clinical Trials

Purpose of review

There is an established association between hypertension and increased risk of poor cognitive performance and dementia including Alzheimer’s disease; however, associations between antihypertensive medications (AHMs) and dementia risk are less consistent. An increased interest in AHM has resulted in expanding publications; however, none of the recent reviews are comprehensive. Our extensive review includes 15 observational and randomized controlled trials (RCTs) published over the last 5 years, assessing the relationship between AHM and cognitive impairment.

Recent findings

All classes of AHM showed similar result patterns in human studies with the majority of study results reporting point estimates below one and only a small number of studies (N?=?15) reporting statistically significant results in favor of a specific class.

Summary

Only a small number of studies reported statistically significant results in favor of a specific class of AHM. Methodological limitations of the studies prevent definitive conclusions. Further work is now needed to evaluate the class of AHM and cognitive outcomes in future RCTs, with a particular focus on the drugs with the promising results in both animals and human observational studies.

     

Hypervolemia rather than arterial calcification and extracoronary atherosclerosis is the main determinant of pulse pressure in hemodialysis patients

Introduction

Pulse pressure (PP) has been reported as an independent predictor of cardiovascular mortality in hemodialysis patients. In this study, we aimed to investigate association of PP with echocardiographic and vascular structural changes such as atherosclerosis and arterial calcifications in HD patients.

Patients and methods

In this cross-sectional study, 108 chronic hemodialysis patients (49 male, 59 female, mean age: 46?±?13?years) were included. Biochemical analyses, echocardiographic and high-resolution carotid Doppler examinations were done. Aortic wall and coronary artery calcifications were measured with electron beam computed tomography. The degree of carotid artery stenosis was measured at four different sites (communis, bulbus, interna and externa) in both carotid arteries.

Results

PP was strongly correlated with systolic (r: 0.82) and diastolic (r: 0.33) blood pressure, left ventricular mass index (r: 0.58), left ventricle end diastolic diameter (r: 0.38) and weakly correlated with aortic wall calcification score (r: 0.26) and carotid plaque score (r: 0.27), but not with coronary artery calcification score. Patients with carotid plaque had higher PP than patients without plaque (50?±?16?mmHg versus 44?±?14?mmHg, P?=?0.05). Patients were divided into three groups according to aortic wall calcification score. PP was significantly higher in patients with higher aortic wall calcification (54?±?16?mmHg) than patients with lower aortic wall calcification (44?±?15?mmHg, P?=?0.04). However, on multivariate linear regression analysis for predicting PP, the only significant factor retained was left ventricle end diastolic diameter.

Conclusion

PP was weakly associated with large vessel calcification and atherosclerosis in hemodialysis patients. The bulk of the effect on PP seems to be due to hypervolemia.     

Clinicopathological characteristics and treatment outcomes of adult patients with paratesticular rhabdomyosarcoma (PRMS): A 10-year single-centre experience

Introduction:

We report our experience with 8 consecutive adults treated for paratesticular rhabdomyosarcoma (RMS) at a single institution between 2000 and 2010.

Methods:

After primary surgical excision, 7 patients were classified into group I according to the Intergroup Rhabdomyosarcoma Study Group (IRSG) Postsurgical Grouping Classification, and 1 patient into group IIB. Retroperitoneal node dissection was not a required staging procedure. Adjuvant chemotherapy was administered to 7 of the 8 patients. No additional radiotherapy was administered.

Results:

The median age at diagnosis was 24 years (range: 18–60). Embryonal histology was the most common (75%) subtype. During follow-up, 3 patients experienced local relapse and 5 distant relapse. The median progression-free and overall survival times were 17.0 ± 9.9 months (range: 5–31) and 27.3 ± 1.3 months (range: 16–58), respectively.

Conclusion:

Paratesticular RMS is an uncommon malignancy in adults. We confirm that patients with localized paratesticular RMS may have different prognoses. Retroperitoneal lymphadenectomy can be avoided as a treatment for paratesticular RMS after radical inguinal orchiectomy.     

Incidence of Appropriate Shock in Implantable Cardioverter-Defibrillator Patients With Improved Ejection Fraction

BackgroundHeart failure with recovered ejection fraction (EF) is a recently described clinical entity. There is insufficient information on the management of implantable-cardioverter defibrillator (ICD) patients with improved EF at generator replacement.Methods and ResultsWe examined the incidence of appropriate shocks in 91 consecutive patients with ICDs for primary prevention of sudden death who underwent generator replacement. Improved EF was defined as both EF >35% at generator replacement and increase in EF by ≥10% since original implantation. Patients were 70 ± 11 years old, and 76% had ischemic cardiomyopathy. At generator replacement, 25 patients (27%) had improved EF (0.49 ± 0.08 vs 0.31 ± 0.07 at baseline; P < .0001). Over 6.2 ± 2.2 years of follow-up after original implantation, 9 patients (36%) with improved EF versus 19 (29%) with unchanged EF had appropriate ICD shocks (P = .51). Incidence of appropriate ICD shocks was similar between the two groups before (P = .90) and after (P = .97) generator replacement. Of the 9 improved EF patients with appropriate shock, 4 had shocks before generator replacement, 2 had shocks before and after generator replacement, and 3 patients, who never had shocks before, had their first shock after generator replacement.ConclusionsSome ICD patients whose EF improves to >35% at generator replacement remain at risk for appropriate ICD shocks.