Ali Taher

..Here, we review the major clinical complications in thalassemia intermedia and suggest some therapeutic strategies based on retrospective clinical observations...

..8%), and diarrhea (n = 6; 3.6%). This is the first randomized study showing that iron chelation with deferasirox significantly reduces iron overload in NTDT patients with a frequency of overall adverse events similar to placebo...

..Nonprogressive increases in levels of serum creatinine have also been observed after exposure to some iron chelators. Longitudinal studies are needed to understand the true burden of renal dysfunction in patients with β-thalassemia...

..This review describes the current state of the art for medical management of TI, with particular focus on the roles of splenectomy, transfusion, and iron chelation therapy...

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..abstract:..

..This has important implications for patients who are heavily transfused and may require higher doses to reduce body iron burden...

..We also discuss the recommendations for thrombosis prophylaxis in these patients...

..Recommendations for thrombosis prophylaxis in these patients are also discussed...

..This study provides evidence that calls for prospective evaluation of the roles of transfusion, iron chelation, and hydroxyurea therapy in TI patients...

..Patients reported greater satisfaction and convenience, and lower impact on daily activities, with deferasirox than with previous ICT. This may help improve adherence to lifelong ICT in iron-overloaded beta-thalassemia patients...

..16 was found between eligibility for VTE prophylaxis on one hand and its application in practice (P < 0.001). Risk factors for VTE and eligibility for VTE prophylaxis are common, but VTE prophylaxis and guidelines application are low...

..Better understanding of the need to assess and treat iron overload in both transfused and non-transfused TI patients is clearly required...

..36; P = 0.002) and serum ferritin (Pearson correlation 0.421; P < 0.0001); with higher levels observed in splenectomised patients. NTBI assessment has potential as a simple reliable approach to determining iron status in TI...

..The study aims at studying the ophthalmologic status in a group of thalassemia patients...

..Hb increase was evident in 47.0% of values. A clinically relevant increase in Hb (>0.5 g/dL) occurred in 23.9% of values. VPA is associated with a change in total Hb level that correlates inversely with baseline Hb levels...

..Phase II/III studies have shown that oral deferasirox 20-30 mg/kg/d reduces iron burden, depending on transfusional iron intake...

..05). Liver iron levels at the end of the 2-year study ranged from 1.0 to 30.9 mg/g dry weight, 3 of the patients having levels above 15 mg/g...

..Brain involvement has not been widely studied in TI, although limited reports confirm a low incidence of overt stroke and high incidence of silent brain infarcts...

..Until solid evidence-based guidelines are available, individualised treatment should be entertained...

..This risk should be seriously taken into consideration when prescribing ticlopidine. G-CSF may be helpful in the treatment of ticlopidine-induced aplastic anemia...

..However, the limitations of this treatment have stimulated the design of alternative orally active iron chelators...

..The pleural effusion was controlled by pleurodesis using tetracycline. We review the literature on this uncommon complication of beta-thalassemia intermedia and discuss the options for diagnosis and treatment...

..Optimal iron overload assessment and monitoring is a key element in the development of improved strategies of iron chelation therapy that can be tailored to meet the patient's specific needs...

..4 ± 5.2 mg/kg/d and significantly reducing LIC in patients with LIC ≥ 7 mg Fe/g dw at a mean dose of 25.7 ± 4.2 mg/kg/d, along with a manageable safety profile...

..A significant relationship between pulmonary thromboembolism and pulmonary hypertension in these patients was noted...

..44.3%). Doses ≥ 30 mg/kg/d were generally required because of high transfusional iron intake and high baseline serum ferritin levels, highlighting the importance of administering an adequate dose to achieve net negative iron balance...

..This study evaluates whether iron overload in patients with β thalassemia intermedia is associated with morbidity...

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..This report is a pilot study assessing the antitumor efficacy and safety of this regimen as first line therapy for advanced breast cancer patients...

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..Main side-effects were skin reactions (DFX alone), nausea and arthralgia (combined therapy). As chelation therapy, the combined protocol was as effective as DFX five times weekly...

..Our data will be compared with other groups using the same regimen for the management of advanced breast carcinoma...

..The latter's V617F mutation does not seem to play a role in this hematologically important clinical entity...

..Due to the increased risk of thromboembolic events, the rationale for splenectomy should perhaps be re-assessed and the role of transfusion therapy for the prophylaxis of thrombosis, among other complications, be evaluated prospectively...

..The aim of the study was to document whether these patients suffer from a polyneuropathy and to determine any contributing factors for the development of neuropathy...

..3%. TTP remains a fatal disease. A high index of suspicion should, therefore, always be present. Treatment options should be further developed and patients should directly be referred to tertiary care centers...

..05). Transfusion requirements rose by 34% in the combination arm (P=0.08). Peginterferon/ribavirin was effective in thalassaemics with HCV and moderate iron overload...

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..Although the KD lesions persisted, they became softer and less nodular. As for the MF lesions, they flared transiently and then exhibited a sustained improvement over a follow-up period of 1 year...

..This study aims at assessing how much house-staff in a tertiary care setting are implementing these new recommendations in the hope that through quantitative analysis of the deficiency we would be able to identify areas of weakness...

..The mortality rate was 100 percent. CONCLUSION: TA-GVHD is a serious complication with very high mortality. Effective prevention guidelines should be established in Lebanon including irradiation and the creation of a central blood bank...

..This report is the first of its kind from Lebanon that analyzes the distribution of this mutation among a series of referred cases to a major tertiary referral center...

..Hypertransfusion seems to be a promising treatment method that should be recommended as a first-line approach or as an adjuvant therapy to other methods...

..In a minority, higher doses of deferiprone or a combination with desferrioxamine would be needed to lower liver iron below 15 mg/g...

..In addition, the high levels of HbF in patients with CAR haplotypes did not seem to ameliorate the severity of symptoms, suggesting that genetic factors other than haplotypes are the major determinants of increased HbF levels in Lebanon...

..In this study, we describe, to the best of our knowledge, the first report of LV only associated with sickle cell trait with significant improvement on aspirin...

..A high index of suspicion is needed for the early recognition and treatment of these conditions...

..In this paper, we report on a patient with advanced breast cancer who attained complete response of 14 months duration to a cisplatin/vinorelbine combination after progression during treatment with paclitaxel and doxorubicin...

..The purpose of this study was to look at the prevalence of these antibodies in patients with RVO disease and no conventional risk factors. We specifically examined how APAs may affect the course of this disease...

..This and stringent infection-control measures are necessary steps to limit the spread of HBV, HCV and perhaps HGV to thalassaemics...

..IgM and IgG anticardiolipin (aCL) were both positive upon admission. The IgM aCL antibodies were still positive 6 weeks later, which suggests their role as a predisposing factor of deep vein thrombosis...

..The five most common mutations were each found on two different haplotypes, and most linkages were as previously reported in other Mediterranean populations, with a few exceptions, also showing some clustering...

..4). This suggests that prophylaxis reduces the risk of arthropathies, the number of future hospital visits and orthopaedic surgeries, and is thus the optimal modality of treatment for patients with severe haemophilia...

..Pericarditis due to C. fetus has rarely been reported. A high index of suspicion is essential to recognise this organism, because of its special microbiological characteristics...

..She remains in complete remission 8 months following the discontinuation of treatment. This is the second reported case of successful treatment of B-cell PLL with rituximab...

..Our patients' clinical course is described. We also review the English literature for patients with this rare mutation...

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..Review of the previously reported cases of pregnancy-related TTP and the current treatment options for this rare condition are discussed also...

..Earlier studies showed that it sees about one third of all cancer cases in Lebanon...

..To report a case of proximal occlusion of 2 major cerebral vessels associated with moyamoya network circulation that manifested by spontaneous intraventricular hemorrhage...

..The use of this technique, along with anticoagulation and if needed pharmacological immunosuppression, should be considered for the acute management of patients who present with this life-threatening condition...

..Despite a high maternal morbidity rate, the perinatal outcome is acceptable when pregnancy progresses beyond the first trimester...

..These issues and our recommendations for handling them are discussed...

..Our case suggests that a thrombophilia work-up may be warranted in BD patients, especially young women before they are prescribed oral contraceptives...

..This topic is gaining more and more importance for both surgeons and internists (especially Hematologists) and we, herein, present a general review of the published literature as an update on the subject...

..Later, while on anti-tuberculous treatment, he developed pancytopenia and bone marrow aspirate showed large macrophages with increased phagocytosis of mature and immature blood elements typical of hemophagocytic syndrome...

..This article will highlight modern management of SCD and its impact on the lives of affected patients...

..The detection of such abnormalities may have major practical consequences for the long-term management of these patients to prevent further thrombotic episodes...

..Although off-label use of rFVIIa at AUB MC was supported by published reports, and associated with few complications, guidelines are required to control use of this medication...

..The introduction of new oral iron chelators and better iron overload quantitation methods will most likely modify this picture, and a follow-up study will examine their impact...

..Evaluation of a larger sample of patients over a longer period of time is needed in order to confirm the favourable results obtained in this study...

..This study provides information about the types and distribution of beta-thalassemia mutations within each religious group and geographic region, which is essential for the implementation of screening and prevention programs...

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..On reviewing the literature, only four cases of thalassemia and lymphoma have been reported worldwide, thus making our case the fifth report of this rare combination of diseases...

..It is unknown whether L371V is a private mutation limited to this family, or will prove to be a common mutation within the Lebanese population...

..The dramatic response to therapy with plasma exchange, a treatment often effective in TTP, suggests a similarity in pathophysiology of micro-vascular occlusion and multi-organ failure in sickle cell disease...

..The clinical characteristics of a patient diagnosed with IgM myeloma, and his excellent response to treatment are reported here...

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..We report a case of a 47-year-old woman diagnosed with ovarian cancer who presented with abdominal distention and thrombocytopenia. Her clinical course and the mechanisms of thrombocytopenia are discussed...

..The patient presented with vaginal bleeding and was found to have wide spread metastasis. Endometrial metastasis in patients with signet-ring breast cancer usually denotes a poor prognosis...

..Therefore the aim of this study was to describe the experience of a tertiary care center in thrombophilia work up among patients diagnosed with VTE...

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..Lebanon is known to harbor sickle cell disease, thalassemia and sickle beta-thalassemia hemoglobinopathy patients along with a documented high prevalence of genetic thrombophilia mutations...

..Approximately 15,000 new cases of myelodysplastic syndromes (MDS) are expected in the United States each year...

..Toxicity of DFP was mild to moderate and acceptable; most commonly, transient arthropathy and nausea/vomiting were observed. Thus, combination therapy has shown to be effective in reducing iron overload in thalassemia patients...

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..Chronic anemia and the tissue hypoxia it induces impair free fatty acid oxidation and ATP production in myocardial cells. The use of L-carnitine, a butyric acid derivative, may help overcome some of these defects...