Hemoglobin Beograd (B121 Glu-Val) is a rarely reported hemoglobin variant. It was first reported in Turkey in 1984. This report is a further observation of this variant in a 22-years old Turkish man.

Beta-thalassemia is among the most common genetic disorders in the world and in Iran, with widespread occurrence. A cross-sectional study on 648 beta-thalassemia patients in Shiraz, Iran was carried out to determine the demography of beta-thalassemia major in Shiraz city, Fars province, Iran and also the rate of consanguinity and the significance of pre-marriage counseling in decreasing familial marriages and consequently preventing this autosomal recessive genetic disease. All interviewed patients had thalassemia major and their age, sex, and the consanguinity between parents were recorded. 40.6% of beta-thalassemia patients were outcomes of first-cousin marriages. Comparison of the percentages of familial marriages (consanguinity) between parents of beta-thalassemia patients and a sample of normal population, revealed a statistically significant difference (p< 0.00001). A nonstatistically significant difference was observed between male (53.5%) and female (46.5%) thalassemia patients. Comparison of data with the situation in 22 years ago revealed a 16.4% decrease in familial marriages among thalassemic families, however, more education and awareness of young women and men about the increased risk of beta-thalassemia after familial marriage through pre-marriage counseling is still necessary.

Nicotine is used in different forms including smokeless tobacco. A special kind of smokeless tobacco also known as Maraş powder (MP) is widely used in southeastern region, especially Kahramanmaraş, Gaziantep and other southeastern cities of Turkey. The aim of this study was to investigate the effect of nicotine on hematological parameters in MP users. Ninety-two MP users from Kahramanmaraş and its environs and sixtyeight healthy controls who did not use MP were included in the study. We measured haematological parameters in the blood samples of MP users and controls. Our results showed that while iron and WBC levels were higher in MP users than the controls (p< 0.001), monocyte and platelet counts were lower (p< 0.05 and p< 0.001, respectively). Increased leukocyte counts in MP users may be an indicator of the present inflammatory events in various tissues. So, we assume that MP, because of either high nicotine content or high tobacco-specific nitroso amines levels (TSNA), causes chronic inflammatory changes in various cells, organs and systemic circulation.

Our aim in this study was to investigate the effects of transfusion on pulmonary function tests (PFT) in patients with thalassemia. A total of 43 patients (34 major, 6 intermedia and 3 S/B thalassemics), 21 females and 22 males aged 6 to 29 (mean ± SD: 13.82 ± 4.96) years participated. PFT was performed using a spirometry (ST-250) programmed with European Community for Coal and a Steal (ECCS) equations. Transfusion did not effect the PFT parameters at the pre- and posttransfusion period and but the type of pulmonary function disorders partially changed after transfusion in our patients.

Apoptosis is a feature commonly seen in tumors; it is an actively regulated cellular process that leads to cell death, in fact the ability to resist apoptosis may seem to offer an advantage to a growing tumor by slowing down the cell loss rate. The present study is a retrospective study aiming at evaluating the Fas/Apo-1, CD95 and bcl-2 oncoproteins immunohistochemically in 30 bone marrow tissue specimens invaded by non- Hodgkin lymphomas (stage IV), 60% of cases were under chemotherapy regimen, while the remainders were still. The study included the significance of the apoptotic inducer Fas/CD95 and apoptotic inhibitor bcl-2 immunoreactivity in relation to a number of clinicopathological variables including age, sex, pattern of malignant cell infiltration in bone marrow, type of malignant cells disseminated and the effect of chemotherapy in relation to apoptotic changes. Fas/CD95 immunoreactivity was positive in 23 cases; 76.7%, positive immunoreactivity was significantly associated with the chemotherapeutic effect (p= 0.0002). On the other side bcl-2 immunoreactivity was positive in 7 cases; 23.3%. A significant association was found between bcl-2 positive immunoreactivity and sex (p= 0.03), pattern of malignant cell infiltration in bone marrow (p= 0.02) and lack of therapy effects (p= 0.0004). As a conclusion (1) Apoptosis is a common feature in non-Hodgkin ’s lymphomas as confirmed by the high incidence of the apoptotic inducer Fas/CD95 positive immunoreactivity; (2) Fas/CD95 and bcl-2 as a tumor markers may work as a useful aid in establishing an apoptosis interpretation in cases of non-Hodgkin ’s lymphomas (stage IV); (3) Fas/CD95 expression translate the chemotherapeutic effects on malignant cells in non-Hodgkin ’s lymphomas; meanwhile the apoptotic inhibitor bcl-2 expression is an independent negative prognostic marker that has been shown to confer resistance to apoptosis.

Primary biliary cirrhosis (PBC) is characterized by a continuous T-lymphocyte mediated attack on small intralobular bile ducts, with their gradual destruction. Patients with PBC often exhibit concomitant autoimmune conditions, and autoimmune hemolytic anemia (AIHA), that is idiopathic in 50% of cases, has rarely been associated to PBC. Ursodeoxycholic acid (UDCA) has been considered the main treatment to PBC patients through a decrease in the detergent effect of endogenous bile acids, concomitantly reducing the hemolytic process. We report the case of a female patient with AIHA complicating PBC, treated with short course prednisone and UDCA, with good response.

An 80-years-old-man with adult polycystic kidney disease and familial multiple myeloma that is complicated with massive pericardial effusion and dilated cardiomyopathy during the course of the disease is presented. Although no definite single genetic disorder is described, multiple myeloma cases may be seen in certain families. Environmental factors are also blamed in the etiology. Multiple myeloma may be complicated by myocardial and pericardial involvement, diagnoses of which are possible only during postmortem examination in some cases.