Oxidant status and antioxidants play important roles in anemias. The present study was conducted to investigate the oxidant-antioxidant status in iron deficiency anemia (IDA), and to evaluate the antioxidant effect of vitamin E in IDA treatment. Ten patients with IDA aged nine months were given only iron treatment, whereas another 10 patients were administered both iron and vitamin E. The complete blood count, plasma malonyldialdehyde (MDA) level, erythrocyte superoxide dismutase level, and the serum vitamin E level, both before and within the treatment phases were examined. The reticulocyte count at the first week of treatment was found lower in the vitamin E-treated group. The mean corpuscular volume (MCV) was found higher in the vitamin E-treated group at the end of therapy. The malonyldialdehyde levels of the group treated with vitamin E were found lower during treatment. These results suggest that iron administration in IDA treatment may stimulate lipid peroxidation, and that vitamin E supplied with iron may reduce the MDA production. The hematological indications of the findings of our study are that the reticulocyte response develops earlier and the microcytosis recovery occurs more rapidly in the vitamin E-administered group in comparison with the group treated with iron only.

Development of factor VIII inhibitors remains the most serious and life-threatening complication of hemophilia therapy. The aim of this study was to determine the prevalence of inhibitor development in Turkish patients with hemophilia. Totally 1226 patients were screened [HA: 1057, HB: 105, von Willebrand’s disease (vWD): 64]. Ages ranged from 1 to 55 years (mean: 16.5 years). Sixty-two percent of patients (657/1057) were severe hemophilia. This study showed that inhibitor prevalence in Turkish hemophiliacs exposed to factor concentrates and fresh frozen plasma (FFP) is 11.2% for all HA and 15.8% for severe HA versus 1.9% for HB after eliminating transient inhibitors. Totally 122 patients were found inhibitor positive [high responder (HR) inhibitor= 60 and low responder (LR) inhibitor= 59 for HA/2 LR for HB/1 LR for vWD]. Thanks to this project, patients with inhibitor development can be treated with specific products such as recombinant factor VIIa or activated protrombin complex concentrates for their bleeding episodes or in their elective operations.

Hemophilia A is an X-linked bleeding disorder resulting mostly from heterogeneous point mutations in the factor VIII (F8) gene. Small/large gene deletions, insertions and gross gene rearrangements underlie the molecular pathogenesis of the disease. Two large inversion mutations due to intrachromosomal recombinations between inverted repeats found in intronic sequences and upstream regions of the F8 gene result in severe hemophilia A. The intron 1 inversion mutation is responsible for about 2% of the cases in various populations. Herein, we report the establishment of the long polymerase chain reaction (PCR) intron 1 inversion mutation detection in our laboratory and a similar frequency of 1-2% among Turkish patients.

We evaluated the effects of pentoxifylline and indomethacin and heparin in a rabbit model of disseminated intravascular coagulation (DIC) induced by infusion of 100 μg/kg/hour of Escherichia coli endotoxin lipoplysaccharide (LPS) for 6 hour. Heparin, indomethacin, pentoxifylline or saline were administered simultaneously with LPS. In addition, a control group was formed which was administered only saline. Hemostatic markers at 0, 1/2, 2, and 6 hour as well as histopathologic changes in the organs and the mortality at 24 hour were determined. The infusion of LPS caused a severe impairment in hemostasis and fibrin accumulation in the pulmonary vasculature. Heparin significantly improved hemostatic impairment and reduced the fibrin accumulation in the pulmonary vasculature. Pentoxifylline and indomethacin had no significant effect on DIC, except that pentoxifylline prevented the decrement in platelet count slightly (p< 0.05). None of the drugs, including heparin, had any effect on mortality. As a result, the prevention of the synthesis of only one cytokine or autocoid is not considered enough to control the results of endotoxemia.

Cytokinesis plays an important role in the etiology of multiple myeloma. The transforming growth factor (TGF) beta 1 levels in 82 sera from 60 patients with multiple myeloma were analyzed by ELISA. Fourty one sample were obtained before treatment from newly diagnosed patients, 22 after treatment from the same patients and 19 from relapsed/refractory patients. Serum median TGF level of newly diagnosed patients was 769.5 ng/mL (126-1853), and the relapsed/refractory patients had similar levels. TGF levels after chemotherapy were not different between patients that reached plateau phase and those who remained refractory. We found a negative correlation between TGF and C-reactive protein and blood urea nitrogen and a positive correlation between TGF and hemoglobin level in newly diagnosed patients. After treatment, it was determined that TGF levels at diagnosis were higher in patients who reached plateau phase than in the refractory patients. Elevated serum TGF concentration at diagnosis in multiple myeloma patients may be a favorable predictor of response.

Hemophilic pseudotumors are a rare but serious complication. They are more common in the weight-bearing
joints. These lesions could be treated by conservative measures using factor VIII cryoprecipitate or by radiation
therapy as an alternative to surgery with good results. We present a case of chronic hemophilia with
pseudotumor of the right knee joint who was treated with low dose external beam radiation with fair results.

A one-year-old infant presented with recurrent perianal abscesses and pneumonia, and was diagnosed with chronic neutropenia. Treatment with granulocyte colony-stimulating factor (G-CSF) was initiated, and thrombocytopenia was detected three weeks later. The drug was discontinued but the patient’s platelet count did not improve. A short course of high-dose methylprednisolone was administered, and both the platelet and neutrophil counts returned to normal. Based on this response, in order to maintain the neutrophil count the steroid treatment was continued for one year with tapered doses. At the time of writing, the patient had been doing well for 13 months without treatment. This report highlights that G-CSF treatment for severe chronic neutropenia in a child may be associated with thrombocytopenia.

Extranodal nerve system involvement is seen in approximately 5% of the patients with non-Hodgkin’s lymphomas. It causes as primary or secondary infiltration of brain, leptomeninges, or peripheral nerves and the signs of spinal cord compression. Here we present a 67-year-old woman with advanced stage diffuse large B-cell lymphoma who developed foot-drop due to lumbosacral plexus involvement.