The role of the prothrombotic mutations in pediatric thrombosis are being investigated. Mutations in the factor V gene (FV G1691A and FV A4070G) leading to activated protein C resistance, the main pathological condition in thrombosis and prothrombotic mutations contributing to thrombosis have been identified. The aim of this study is to reveal the role of FV A4070G mutation in pediatric thrombosis. 314 patients with thrombosis at pediatric age including also 111 stroke patients and 127 healthy controls were included to the study. The FV A4070G mutation was evaluated independently and also the combined effects with other prothrombotic mutations were also investigated. In pediatric stroke group FV A4070G was found not to be a risk factor with an OR of 1.04 (CI 95%: 0.56-1.93, p= 0.884). Further studies are needed to study the role of FV A4070G with other risk factors belong to HR2 haplotype.

Differences in haemostatic parameters have been reported among smokers and non-smokers. However the relationships of these parameters with other risk factors of cardiovascular diseases have not been fully determined in Nigerians. We therefore aimed at assessing the relationship between fibrinogen, factor VII (FVII), age, body mass indeks (BMI), systolic blood pressure (SBP) and diastolic blood pressure (DBP) in smokers and non-smokers living in an Urban Nigerian city. We studied 104 male Nigerians grouped into non-smokers (n= 74) and smokers (n= 30). Fibrinogen was measured using the clot weight method of Ingram and FVII was assayed by the bioassay method. Mean fibrinogen and FVII were found to be higher in smokers than non-smokers. There was no relationship between FVII and age (r= -0.0458) in non-smokers and a weak association was found between FVII and age in smokers (r= 0.3191). Both SBP and DBP were significantly associated with BMI in smokers (r= 0.6332, p= 0.0002) for DBP and (r= 0.4869, p= 0.0064) for SBP. Fibrinogen was only associated with DBP in non-smokers (r= 0.3273, p= 0.0047). FVII and fibrinogen were found to be higher in smokers compared to non- smokers, the difference was not statistically significant (p= 0.1965). The strength of the association of BMI and fibrinogen with blood pressure is higher for DBP in Nigerians. Extensive population studies should be conducted on smokers and non-smokers to confirm these associations.

The aim of this study was to determine the effect of high-dose methylprednisolone (HDMP) on lymphocyte subtypes, CD4/CD8 ratio and clinical efficacy of the treatment in children with acute immune thrombocytopenic purpura (ITP). The study consisted of 21 children (aged between 1.5-14 years) with ITP treated with HDMP for 7 days. Absolute lymphocyte count, CD4+ and CD8+ T lymphocyte levels were examined on peripheral blood and CD4/CD8 ratio was calculated before and after HMTP treatment (on 0 and 8th days) in all subjects. There was no statistically significant difference for age and sex between the study and the control group. A significant reduction was observed in the percentage of CD4+ lymphocyte (39.0 ± 7.5% vs 29.3 ± 8.1%, p= 0.001), CD8+ lymphocyte (27.1 ± 7.2% vs 23.7 ± 8.3%, p= 0.03), CD4+/CD8+ (1.5 ± 0.5 vs 1.3 ± 0.4, p= 0.02) and the absolute number of CD4+ lymphocyte count (1694.99 ± 1019 vs 1199.12 ± 612, p= 0.038). These findings indicated that HDMP treatment may cause a decrease in the percentage of CD4+ and CD8+ T-lymphocyte and ratio of CD4+/CD8+ lymphocyte in patients with acute ITP. We suggest that the effectiveness of steroids may depended upon the suppression of CD4 T-lymphocyte and sequential monitoring of circulating lymphocyte subtypes may be used to predict the clinical effects of steroid treatment.

We investigated the association of cytokine gene polymorphisms with the development of chronic myelogenous leukemia (CML) and whether there is an association between gene polymorphisms Th1 and Th2 or regulatory- type cytokines and CML. Thirty patients with CML and 60 healthy controls were enrolled in this study. All genotyping (TNF-α, TGF-β, IL-10, IL-6, and IFN-γ) studies were performed using sequence-specific primers PCR (PCR-SSP). Frequencies of IL-10 (-1082, -819, -592) GCC/ATA (p= 0.009) and IFN-γ; +874 T/A (p= 0.037) polymorphisms were significantly greater in the patients with CML. In contrast, significantly lower frequencies of the IFN-γ A/A (p= 0.004) genotype were observed in patients with CML compared with controls. The results suggest that the IL-10 GCC/ATA and IFN-γ T/A polymorphisms are potential risk factors, and that the IFN-γ A/A polymorphism is a protective factor, for CML in this study group.

Posttransplant lymphoproliferative disorder (PTLD) is a serious complication of organ transplantation, with a reported incidence between 0.8% to 32%. Herein we retrospectively analyzed the patients who diagnosed as PTLD in Akdeniz University. Within the 782 (773 renal and 9 heart) transplant recipients six patients were diagnosed as PTLD (diffuse large B-cell lymphoma). Five of them had renal, one had cardiac transplantation. Three patients were diagnosed within the first year of transplantation. Five patients had abdominal disease one had central nervous system involvement. All patients had positive Epstein-Barr virus (EBV) and cytomegalovirus (CMV) IgG at the time of diagnose. EBV-DNA with polymerase chain reaction (PCR) was found to be negative in five patients. Only one patient was survived after the diagnosis of PTLD. In conclusion, even with treatment the mortality rate is high in patients with PTLD. In order to decrease the incidence of PTLD and related mortality, the risk factors should be evaluated with multicenter studies.

Acquired amegakaryocytic thrombocytopenia (AAT) is a rare disease characterized by severe thrombocytopenia due to selective reduction or absence of megakaryocytes in the bone marrow. More commonly, patients with AAT have additional hematologic abnormalities such as macrocytosis or dyserythropoiesis, abnormalities which may predict progression to aplastic anemia or myelodysplasia. A 52-year-old female was admitted to hospital with mucosal and gingival bleeding. Megakaryocytes were not seen on the bone marrow aspiration and biopsy. AAT was diagnosed. Although she was treated with immunosuppressive therapy including prednisolone and cyclosporine, her disease progressed to myelodysplastic syndrome. She died at the third month of diagnosis because of cerebral bleeding.

Osteonecrosis is an uncommon complication of acute lymphoblastic leukemia. One of the risk factor is high-dose corticosteroid therapy. The highest incidence of osteonecrosis is observed in children 9 to 18 years old at diagnosis and it is a rare condition below 5 years of age. We describe a 4 year-old child with acute lymphoblastic leukemia and complaints of progressive bone pain and walking difficulty who developed osteonecrosis and bone fracture after two remission induction chemotherapy.