Background and Objectives:In this study, we investigated the T helper (Th) to T cytotoxic (Tc) ratio in children suffered from hemophilia disease. 80 diagnostic subjects referred to Ali Asghar hospital of Zahedan city, Zahedan-Iran, were selected and the ratio of these cells were analyzed by analytic descriptive methods.Method of study:Two mls of EDTA anti coagulated whole blood was collected. Immunophenotyping of lymphocytes count was carried out by FACS analysis using a double CD4 and CD8 kit. The mean ± SD of absolute numbers of CD4 and CD8 lymphocytes/ml was calculated and the ratio of CD4/CD8 was evaluated by statistical method.Results:We found 100% of patients were type A hemophilia. Among of them 66 (82.5%) were male. The mean age was 15±3.51 years. 12 (15%) of them had mild disease intensity and 68 (85%) had sever disease intensity. The ratio of CD4 and CD8 was obtained between 0.45 and 1.44 with mean1.79 ±0.78 and correlation with the gender and disease intesivity was 0.095 and 0.019 respectively.Conclusions:The results showed that the ratio of T helper (Th) to T cytotoxic (Tc) has significant correlation with

Objectives: Congenital prothrombin (factor II) deficiency is an inherited rare bleeding disorder with an autosomal recessive manner. The prevalence of this disorder is about one in 2000000 people in general population, but it is more common in areas with a high rate of consanguinity. To date, there is no report on the absence of prothrombin, which is a life-threating disorder. Considering the importance of factor II in body homeostasis, this study aimed to find any possible mutation of coagulation factor II codons in patients with inherited factor II deficiency in southeastern Iran. Materials andMethods: This study was conducted on 12 patients with inherited deficiency of prothrombin. Early diagnosis was based on clinical symptoms, laboratory evaluation, and family history. Then, the function level of prothrombin was measured, the initial diagnosis of disease was confirmed, and polymerase chain reaction (PCR) analysis was performed. Finally, gene sequencing and genotyping of factor II was done. Results: Molecular analysis indicated a point mutation in exon 7 in three patients and a frameshift mutation in exon 14 due to addition of a thymine base at position 1760-1761 in one patient, both of which have been reported for the first time. Conclusions: Molecular methods performed on patients from Southeastern Iranian population in terms of coagulation factor II deficiency revealed a substitution mutation in exon 7 in three patients and a frameshift mutation in exon 14 in one patient, both of which were reported for the first time. Considering the significant difference between the clinical symptoms of the present study and previous studies, probably the type of mutations reported in this study (for the first time) caused these clinical symptoms, but statistical studies did not show any relationship between the type of mutation and the occurrence of clinical symptoms. And it needs more investigations on more patients, with a larger population.