Cancer-Associated Thrombosis

Thrombosis is a common complication in patients with cancer and it is estimated that about 20% of patients with cancer experience venous thromboembolism (VTE). This complication is associated with high rate of morbidity and mortality and is sometimes the first manifestation of an occult cancer. The risk profiles and markers involved in cancerassociated thrombosis share similarities with inflammation-induced atherosclerosis and thrombosis. The type of cancer, chemotherapy, surgery, central venous catheters, pre-chemotherapy platelet and leukocyte count are associated with high risk of VTE in cancer patients. Landmark studies demonstrated that effective prophylaxis and treatment of VTE reduced morbidity and increased survival. Low-molecular-weight heparin (LMWH) is preferred as an effective and safe means for prophylaxis and treatment of VTE. It has largely replaced unfractionated heparin and vitamin K antagonists. The advantages of LMWH include increased survival and quality of life, decreased rate of VTE, low incidence of thrombocytopenia. New guidelines for prophylaxis and treatment are now available and prophylaxis is recommended in hospitalized cancer patients and patients undergoing major surgery. Treatment with LMWH should be considered as the first line of therapy for established VTE and to prevent recurrent thrombosis in patients with cancer

Quality of life: Transfusion dependent thalassemia vs non-transfusion dependent thalassemia

Although the improvements in the treatment and management of thalassemia patients in new years lead to the improved survival and quality of life (QOL) in this group of patients, QOL is still is an important dimension of care in thalassemic patients. WHO defines QOL as “an individual’s perception of their position in life in the context of the culture and value systems in which they live and in relation to their goals, expectations, standards, and concerns”. Thalassemia is a chronic disease needs life-long care with multiple physical, mental and social complications that affect QOL in patients. The most important factors which affect QOL in thalassemia are: effects of the disease on family, skeletal and face changes, frequent blood transfusion and drug infusion, sexual impairment and infertility, heart and liver disease as well as endocrine disorders, anxiety and low life expectancy

Arg77His and Trp187Arg are the Most Common Mutations Causing FXIII Deficiency in Iran

The aim of this study was to review the literature for the genetic mutations causing inherited factoe XIII (FXIII) deficiency in patients from Iran, where the consanguineous marriage is common. Data were collected from 30 patients (18 males and 12 females) with FXIII deficiency, from 26 unrelated families. Data of mutation analysis were obtained from 2 previously published studies. A total of 7 mutations consisting of 5 new mutations and 2 previously reported mutations were identified. Of the 5 novel missense mutations, 2, Arg77His and Trp187Arg, were the most common in Iranian FXIII-deficient patients. In regions like Iran with high rate of consanguineous marriages, the identification of common mutations in disease like severe FXIII deficiency increases the capacity to make a precise screening and diagnosis assays to screen and diagnose families with high risk of FXIII deficiency for prevention of clinical complications in them

Induction of HBF

The role of HbF inducers in ameliorating the pathophysiology and severity associated with hemoglobinopathies like sickle cell disease and thalassemia is well demonstrated. So many studies have focused on explain the pathways that involved in the re-activation of γ-globin gene expression. The HbF inducers are mainly includes chemotherapeutic agents (e.g. 5-Azacytidine and hydroxyurea), short-chain fatty acid derivatives (e.g. butyrates, valporate, sodium phenylbutyrate) and other HbF inducers like stem cell factor and synthetic gene-targeted transcription factors. These HbF inducers have significant effects on improve clinical manifestations of hemoglobinopathies and decreasing transfusion dependency. But regarding some limitations of these agents, more studies for the investigation of new agents with more safety and effectiveness is needed

Characteristics And Prevalence Of Occult Hepatitis B Virus Infection In Patients With Hepatitis C In Iran

Hepatitis B virus (HBV) infection in patients who lack detectable hepatitis B surface antigen (HBsAg) is called occult hepatitis B infection. Such infections have been frequently identified in patients with chronic hepatitis Cliver disease, but their prevalence is not known.Methods: 207 patients with chronic hepatitis C who were HCV -RNA and antiHCV positive were studied for HBV-DNA by PCR, and for HBsAg and anti-HBc by ELISA. DNA was extracted by high pure nucleic acid kit (Roche-Germany). HBVDNA amplification was done with a set of primer directed to the pre-S region. HBsAg and anti-HBc were evaluated by a commercially available ELISA kit (Dade Behring). Results: 23 of 207 patients with chronic hepatitis Cliver disease ( 11.1%) were positive for HBV-DNA (co-infection). Among this group 17 patients (8.2%) were HBsAg negative (occult infection). 8 of 17 patients with occult infection ( 4 7%) were anti-HBc positive and 9 were anti-HBc negative (53%). No significant difference was found in epidemiological and biochemical parameters in patients with HCV alone in comparison with HCV co-infected with occult hepatitis B (p= 0.453 for ALT and p= 0.498 for AST).Conclusion: Occult hepatitis B virus infections occur frequently in patients with chronic hepatitis Cliver disease and may have clinical significance

Initial presentation of childhood leukaemia with facial palsy: three case reports

Acute lymphoblastic leukaemia and acute myeloblastic leukaemia are the most common malignancies diagnosed in children. Facial palsy is an acute peripheral palsy involving the facial nerve and is an unusual presentation of childhood acute leukaemia. We present three cases (a 9-year-old boy, a 14-year-old boy and a 10-year-old boy) of acute leukaemia with initial presentation of facial palsy. It is important for physicians to recognise the neurological manifestations of childhood leukaemia and extensive work-up should be carried out to exclude secondary causes of facial palsy