Spontaneous intracholecystic bleeding in a patient with von Willebrand's disease.
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Biomedical subjects
Publications and source records attributed to Ali Taher.
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BACKGROUND: Although rare, transfusion-associated GVHD (TA-GVHD) is a fatal complication of blood transfusion in which active lymphocytes from the donor attack and destroy recipient organs and tissues. STUDY DESIGN AND METHODS: A search of patient records was carried out at the American University of Beirut-Medical Center, looking for patients who developed TA-GVHD over a 10-year period extending from 1991 to 2001. Relevant information was collected and analyzed. RESULTS: A total of 10 records were found as a result of this search. All were immunocompetent and received fresh nonleukoreduced, nonirradiated blood. The majority received the transfusion at outside periphery hospitals. They received different treatment modalities. 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.
Desferrioxamine (DFX) alone (40-50 mg/kg/d s.c. over 8-12 h, five times weekly) was compared with combined DFX twice weekly and deferiprone (75 mg/kg/d) over 12 months in previously poorly chelated thalassaemia patients. Serum ferritin fell from 5506 +/- 635 microg/l (mean +/- SEM) to 3998 +/- 604 microg/l (P < 0.001; n = 14) in the DFX group and from 4153 +/- 517 microg/l to 2805 +/- 327 microg/l in the combined group (P < 0.01; n = 11). Deferiprone plus DFX produced a greater mean urine iron excretion (1.01 mg/kg/24 h) than iron intake from blood transfusion in each patient. 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.
Thalassemia is a common inherited disease in the Mediterranean region. We here report a mutation new to the Lebanese population: the insertion of a G nucleotide at codons 8/9 [(+G) AAG-TCT (Lys-Ser) --> AAG-G-TCT (beta0)] of the beta-globin gene in a thalassemic patient with a mild phenotype. We discuss the possible factors that play a role in alleviating the severity of the disease in this case.
BACKGROUND: Several reports have described the association between antiphospholipid antibodies (APAs) and retinal venous occlusive (RVO) disease. 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. METHODS: Twenty-four patients with the diagnosis of RVO disease were screened prospectively for APAs. All were free from risk factors for retinal vein thrombosis and other immunologic conditions. Patients were observed for a period of 3 to 12 months. RESULTS: Lupus anticoagulant was negative in all 24 patients. Ten (43%) of 24 patients had anticardiolipin antibodies (ACAs). All patients with ACAs were younger than 45 years of age, with an average age of 33 years. The average age of patients with no ACAs was 66 years. Comparison of the average age of the two groups showed a statistically significant difference. There was no statistical significance between the two groups for development of neovascular disease. Seropositive patients who developed neovascularization had elevated titers for an average of 11.8 weeks versus 3.3 weeks for those who did not have neovascularization. Neovascular complications generally began several weeks after the titers became negative. CONCLUSION: There was a significant prevalence of ACAs in young patients with RVO disease and no associated systemic risk factors. Seropositive patients who developed neovascular disease had elevated titers for more than 6 weeks. However, the role of these transient ACAs in retinal vein occlusion is still not clear and merits further study.
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We describe a case of pulmonary thromboembolism in a 61-year-old woman with beta-thalassemia intermedia and no other risk factors for thrombosis. Thromboembolism was documented by perfusion lung scan. We review the literature on this uncommon complication of thalassemia intermedia and discuss the pathogenesis and treatment options. A significant relationship between pulmonary thromboembolism and pulmonary hypertension in these patients was noted.
The presenting symptoms of malignancies like anemia and splenomegaly in thalassemic patients can be overlooked and considered as complications of thalassemia. Our paper deals with a case of large B cell lymphoma with bone marrow involvement in an old lady with thalassemia intermedia. The patient showed complete response after the second cycle of chemotherapy that consists of cyclophosphamide, vincristine, and prednisone (CVP protocol). However, the patient relapsed with bone marrow involvement shortly after (2 weeks) completion of the sixth cycle of chemotherapy, and she was started on monoclonal antibodies Rituximab. 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.
Acute multi-organ failure syndrome is a rare and life-threatening complication of patients with sickle cell disease. The syndrome appears to be reversed with prompt, aggressive exchange transfusion therapy. It has been attributed to widespread vascular occlusion due to micro-vascular red cell sickling. We present a case of severe multi-organ failure in a patient with sickle thalassemia and mild clinical course, who had clinical and laboratory features consistent with thrombotic thrombocytopenic purpura (TTP). 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.
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BACKGROUND: Patients with thalassemia may complain of numbness and weakness of the lower extremities. 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. PATIENTS AND METHODS: We examined 30 patients with thalassemia major and intermedia, clinically and electrophysiologically. We correlated these findings with demographics, blood status, and treatment and compared electrophysiologic data with 30 age- and sex-matched normal subjects or historical controls. RESULTS: We found that 78% of thalassemic patients suffered from a mild sensory polyneuropathy. The neuropathy seemed to be worse in the intermedia type. Thalassemic patients who received blood transfusions and deferoxamine had better nerve function than those who did not, irrespective of the dose of deferoxamine. The neuropathy was worse for older patients, irrespective of sex. The hemoglobin level, and the fact that some patients underwent splenectomy, did not affect the status of the patients' nerves. CONCLUSION: Patients with thalassemia may suffer from a sensory polyneuropathy especially as they grow older and if they are not optimally treated.
BACKGROUND: Breast cancer is the most common cancer in Lebanese women. Lebanon has no national cancer registry and the American University of Beirut Medical Center (AUBMC) is one of the largest hospitals in Lebanon and has a fully operational cancer registry. Earlier studies showed that it sees about one third of all cancer cases in Lebanon. METHODS: All female breast cancer patients recorded at AUBMC between 1983 and 2000 were evaluated. We used the sex-specific age distribution of 1995 Lebanese Population and Housing Survey to estimate the age-specific incidence of breast cancer in Lebanon. The results were calculated as number and proportion of cases, 10-year age-specific incidence rates, crude rates and age standardized rates (ASR) per 100,000 population. The ASR per 100,000 population was estimated by the direct method with the use of the World Standard Population. RESULTS: Between 1983 and 2000, there were a total of 16421 cancers of which 8007 were in women. There were 2673 female breast cancers, averaging 148 cases per year (Range:94-202). Almost half of cases (49.1%) were in women below the age of fifty. The mean age was:49.8 years +/- 13.9 years. Ten-year age groups distribution showed that 4.7% were below 30 years of age, 16.1% were 30-39 years, 28.3% were 40-49 years, 26.3% were 50-59 years, 16.9 % were 60-69 years, 6.1% were 70-79 years and 1.6% were 80 years of age or older. Twenty-two patients (0.9 %) had their age missing in the records. Overall ASR was 30.6, for a crude rate of 27.7. Age adjusted incidence rate had its peak in women aged 50-59, followed by women 40-49 then 60-69 with values of 96.3, 79.9 and 77.4 per 100,000 respectively. We also noted 19 male breast cancer cases corresponding to 0.7% of the 2692 combined total. CONCLUSIONS: The percentage of women with breast cancer in Lebanon seen at AUBMC in pre-menopausal and younger-aged groups is higher than those reported from western countries. Our results emphasize the need to search for possible environmental, lifestyle and/or genetic risk factors in Lebanon. Our study also shows the importance of implementing early detection and screening programs which, along with high quality mammography and medical care, can have a positive impact on survival, especially in younger-aged women.
Angiogenesis, the formation of new blood vessels from pre-existing ones, is a very well controlled multistep process under physiologic conditions. It involves a balance between several positive and negative regulators. Pathologic angiogenesis, both excessive and insufficient, plays a role in the pathophysiology of many diseases including cancer. It has been proven that tumor growth and metastasis are angiogenesis-dependent. Endothelial cells and tumor cells also provide a mutual support to each other via the secretion of several substances. Targeting tumor blood vessels is a new approach to the treatment of cancer. Clinical trials are currently underway and the results appear to be very promising.
Breast cancer is the most common malignancy in women throughout the world. It is the leading cause of cancer death of women aged > 50 years. Cisplatin and vinorelbine are active as single agents in advanced breast cancer but their combination was not studied before. We conducted three phase II trials using this combination in advanced breast cancer. Hereby, we describe our experience over a 10-year period at the American University of Beirut using this combination in metastatic disease as 1st and 2nd line therapy as well as in the neoadjuvant setting in patients with locally advanced disease. Our data will be compared with other groups using the same regimen for the management of advanced breast carcinoma.