195 research outputs found
Beta-thalassemia intermedia: an overview
No full textThalassemias are inherited anemias characterized by decreased or absent globin chain synthesis and by ineffective erythropoesis. They are classified according to the defective globin chain and to the clinical severity. Beta-thalassemias, which are prevalent in the Mediterranean region, have decreased or absent beta-globin chain synthesis whereas, in alpha-thalassemias, the alpha-chain is affected. Thalassemia major is a severe, transfusion-dependent anemia with significant morbidity and mortality. Thalassemia intermedia are a genetic variant of the beta-thalassemias, which incorporates a less severe anemia than thalassemia major with inefficient erythropoiesis as well as peripheral hemolysis. Leg ulcers, pulmonary arterial hypertension, extramedullary hematopoiesis, and thrombotic events are some of the complications that thalassemia intermedia patients face
Bosutinib: a SRC–ABL tyrosine kinase inhibitor for treatment of chronic myeloid leukemia
No full textFuad El Rassi, Hanna Jean KhouryDivision of Hematology, Department of Hematology and Medical Oncology, the Winship Cancer Institute at Emory University, Atlanta, Georgia, USAAbstract: Bosutinib is one of five tyrosine kinase inhibitors commercially available in the United States for the treatment of chronic myeloid leukemia. This review of bosutinib summarizes the mode of action, pharmacokinetics, efficacy and safety data, as well as the patient-focused perspective through quality-of-life data. Bosutinib has shown considerable and sustained efficacy in chronic myeloid leukemia, especially in the chronic phase, with resistance or intolerance to prior tyrosine kinase inhibitors. Bosutinib has distinct but manageable adverse events. In the absence of T315I and V299L mutations, there are no absolute contraindications for the use of bosutinib in this patient population.Keywords: bosutinib, chronic myeloid leukemia, treatment, review, SRC-ABL kinase inhibitor, clinical activit
Reflections of T2 MRI in 19 thalassemia intermedia patients
No full textAutoimmune thrombocytopenia is a common immune-hematologic complication in non-Hodgkin's lymphomas and may complicate the treatment. We analyzed an original series from our institute as well as published cases of non-Hodgkin's lymphomas (excluding chronic lymphocytic leukemia) associated with autoimmune thrombocytopenia with regard to demographic factors, prevalence in non-Hodgkin's lymphoma subtypes and treatment outcome. The male/female ratio is 1.75. Half of the cases occurred prior to diagnosis of lymphoma. Chemotherapy is the best treatment in many non-Hodgkin's lymphomas patients with autoimmune thrombocytopenia compared with standard treatment of autoimmune thrombocytopenia. Splenectomy is effective in splenic marginal zone lymphoma. Autoimmune thrombocytopenia in patients with non-Hodgkin's lymphomas is potentially life-threatening and difficult to treat
Correlation of liver iron concetration determined by R2 magnetic resonance imaging with serum ferritin in patients with thalassemia intermedia
No full textCorrelations of non transferrin bound iron levels in 74 patients with thalassemia intermedia
No full textLevels of non-transferrin-bound iron as an index of iron overload in patients with thalassaemia intermedia
No full textNon-transferrin-bound iron (NTBI) was evaluated as an index of iron overload in a cross-sectional randomised study in 74 non-transfused patients with thalassaemia intermedia (TI). Mean NTBI (2.92 ± 3.43 μmol/l), serum ferritin (1023 ± 780 ng/ml) and liver iron concentration (LIC; 9.0 ± 7.4 mg Fe/g dry weight) were increased above reference-range levels. Significant positive correlations occurred between mean NTBI and LIC (Pearson correlation 0.36; 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
Hydatid cyst mimicking acute chest syndrome in a sickle thalassemia patient [4]
No full text[No abstract available]BEAUDOIN.A, 1967, ANN BIOL CLIN-PARIS, V25, P199; Sadjadi SM, 2006, PARASITOL INT, V55, pS197, DOI 10.1016-j.parint.2005.11.030; Vichinsky EP, 2000, NEW ENGL J MED, V342, P1855, DOI 10.1056-NEJM2000062234225020
Beta-thalassemia intermedia: An overview
No full text[No abstract available]Aessopos A, 2005, CHEST, V127, P1523, DOI 10.1378-chest.127.5.1523; Aessopos A, 2001, BLOOD, V97, P3411, DOI 10.1182-blood.V97.11.3411; CAPELLINI MD, 2002, HEMATOL J, V65; CAPELLINI MD, 2007, GUIDELINES CLIN MANA, pCH11; Cappellini MD, 2005, SEMIN HEMATOL, V42, pS19, DOI 10.1053-j.seminhematol.2005.01.001; Cappellini MD, 2000, BRIT J HAEMATOL, V111, P467, DOI 10.1046-j.1365-2141.2000.02376.x; Castelli R, 2004, AM J MED SCI, V328, P299, DOI 10.1097-00000441-200411000-00012; Chehal Aref, 2003, Spine (Phila Pa 1976), V28, pE245, DOI 10.1097-00007632-200307010-00024; Dixit A, 2005, ANN HEMATOL, V84, P441, DOI 10.1007-s00277-005-1026-4; Eldor A, 2002, BLOOD, V99, P36, DOI 10.1182-blood.V99.1.36; GIMMON Z, 1982, PLAST RECONSTR SURG, V69, P320, DOI 10.1097-00006534-198202000-00023; Karimi M, 2005, J PEDIAT HEMATOL ONC, V27, P380, DOI 10.1097-01.mph.0000174386.13109.28; Kushner J P, 2001, Hematology Am Soc Hematol Educ Program, P47; Mourad FH, 2003, BRIT J HAEMATOL, V121, P187, DOI 10.1046-j.1365-2141.2003.04240.x; Origa R, 2005, ANN NY ACAD SCI, V1054, P451, DOI 10.1196-annals.1345.051; PERRINE SP, 1993, NEW ENGL J MED, V328, P81, DOI 10.1056-NEJM199301143280202; St Pierre TG, 2005, BLOOD, V105, P855, DOI 10.1182-blood-2004-01-0177; Taher A, 2006, THROMB HAEMOSTASIS, V96, P488, DOI 10.1160-TH06-05-0267; TAHER A, 2006, BLOOD CELL MOL DIS, V27, P12; TAHER A, 2007, ASH ANN M, V110, P3818; Weatherall DJ, 2001, J HEMATOL S1, V86, P18694
Hypovitaminosis D in developing countries-prevalence, risk factors and outcomes
No full textHypovitaminosis D is a prevalent disorder in developing countries. Clinical manifestations of hypovitaminosis D include musculoskeletal disorders, such as nonspecific muscle pain, poor muscle strength and low BMD, as well as nonmusculoskeletal disorders, such as an increased risk of respiratory infections, diabetes mellitus and possibly cardiovascular diseases. In developing countries, the prevalence of hypovitaminosis D varies widely by and within regions; prevalence ranges between 30-90percent, according to the cut-off value used within specific regions, and is independent of latitude. A high prevalence of the disorder exists in China and Mongolia, especially in children, of whom up to 50percent are reported to have serum 25-hydroxyvitamin D levels 12.5 nmol-l. Despite ample sunshine throughout the year, one-third to one-half of individuals living in Sub-Saharan Africa and the Middle East have serum 25-hydroxyvitamin D levels 25 nmol-l, according to studies published in the past decade. Hypovitaminosis D is also prevalent in children and the elderly living in Latin America. Risk factors for hypovitaminosis D in developing countries are similar to those reported in Western countries and include extremes of age, female sex, winter season, dark skin pigmentation, malnutrition, lack of sun exposure, a covered clothing style and obesity. Clinical trials to assess the effect of vitamin D supplementation on classical and nonclassical clinical outcomes in developing countries are needed. © 2010 Macmillan Publishers Limited. 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Complications of β-thalassemia intermedia: A 12-year Lebanese experience
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