IRANIAN JOURNAL OF PEDIATRIC HEMATOLOGY AND ONCOLOGY
Year:2011 | Volume:1 | Issue:2|Papers Count:7

Objective: JAK2 is a non-receptor tyrosine kinase that plays a major role in myeloid disorders. JAK2V617F mutation is characterized by a G to T transverse at nucleotide 1849 in exon 12 of the JAK2 gene, located on the chromosome 9p, leading to a substitution of valine to phenylalanine at amino acid position 617 in the JAK2 protein. Methods: In this study we evaluated RNA from 89 patients with MPNs and statistical analysis done with Mann-Whitney test. The mutation detected by allele specific-PCR(AS-PCR). In addition, 3 samples were sequenced in Millegen company.Results: Using AS-PCR method 26/30 polycythemia vera patients (86%), 8/13 IMF patients (61%), 8/15 ET patients (53%) and none of 31 CML patients were positive for JAK2 V617F mutation.Polycythemia vera patient carrying the mutation displayed higher levels of WBC (p=0.03). Sixteen of 26 JAK2V617F positive patients were female that demonstrate correlation between the presence of a mutant allele and sex. The differences in other groups were not significant.Conclusion: We have shown that a single acquired point mutation in JAK2 is present in virtually most patients with PV and in about half of those with either ET or IMF.However in other study the JAK2V617F mutation has been detected in the vast majority of patients with polycythmia vera (65-95%).  It was less frequent in patients with essential thrombocythemia (23-57%), idiopathic myelofibrosis (23-57%) and chronic myeloid leukemia 19% (3/16 CML Ph-)  Detection of the mutation is helpful in differential diagnosis, prognosis, and prediction of therapeutic response.

Background: Patients who are to receive chemotherapy require careful assessment of liver function prior to treatment to determine which drugs may not be appropriate, and which drug doses should be modified. Following therapy abnormalities of liver function tests may be due to the therapy rather than to progressive disease, and this distinction is of critical importance. Toxicity such as hepatotoxicity could be reduced by L-Carnitine (L.C) without affecting its anti-cancer therapeutic efficacy. The objectives of the present study were to assess the role of L-Carnitine by evaluating the liver functions.Materials and Methods: We performed randomized, double-blind, placebo-controlled study, the number of 64 ALL patients were enrolled in our study. Patients was randomly divided into two groups (each group 32 patients), and using double-blind administration, group A was treated with L-Carnitine and group B with placebo for 90 days. Results: We observed differences in serum AST level 3,7,30 days after chemotherapy (p=0.006, p<0.001, p= 0.001), serum ALT level 7,30 days after chemotherapy (p=0.009, p<0.001), serum ALK-P level after 30,90 days after chemotherapy (p<0.001), Prothrombin time 3, 7, 30 days after chemotherapy (p=0.017, p=0.010, p=0.012).No significant differences were observed in Alb and GGT in either group of patients treated with L-Carnitin or placebo.Conclusion: The benefits of L-Carnitine in comparison with placebo are demonstrated in reduction serum AST, ALT, ALK-P and PT but not in Alb and GGT. These issues deserved to evaluate the value of L-Carnitine in ALL patients.

Introduction: The main function of the fibrinolytic system is to dissolve fibrin clots in circulation. This system is composed of inactive precursor plasminogen which can be converted into plasmin by the proteolytic enzymes like tissue-type plasminogen activator (tPA). Fibrinolytic properties can be found in a variety of medicine plants and they could effectively prevent cardiovascular diseases (CVD). One of these medicine plants is Allium sativum, which was used for its antiplatlet and fibrinolytic effects in patients with CVD (garlic).This study considers to find the fibrinolytic effect of the various concentration of garlic extract and the time of the most effect.Methods: Garlic extracts were prepared using 70% ethanol, and labeling fibrinogen with fluorescent agent to create a labeled clot. Then 10, 25 and 50mg/ml of garlic extract were separately added to plasma environment and finally the labeled clots were inserted. At the end fluorescence intensity of the supernatant was measured. The data were analyzed using SPSS (version 16).Results: The results indicated that garlic extract showed fibrinolytic effect significantly compared with the control group (p<0.05). Various concentrations of garlic extract showed significant different rise in the fibrinolytic activity of blood clot in different time (p<0.05). Desirable result obtained in the lower concentration of (10mg/ml) after 7 hours.  Conclusion: Garlic extract displayed the fibrinolytic activity which concentration and time factors of exposure influenced it but the minimum concentration and the maximum time showed the best result.

Introduction: Major b Thalassemia represents a group of recessively inherited hemoglobin disorder, which is characterized by reduced synthesis of globins chains. Frequent blood transfusions can lead to iron overload, which may result in several endocrine complication especially in the absence of adequate chelating therapy. The objective of this study were to determine the prevalence of hypothyroidism in transfusion dependent thalassemia patients treated in the hematology unit of Shahid Sadoughy hospital and determination of the correlation of hypothyroidism  with the Ferritin level.Methods: This study was cross-sectional study which was performed during one year. Sixty five patients, ages between 1.2 years to 27years old entered to this study (47.6%female and 52.4%male) For all of the patients T3-T4-T3RUP, TSH and ferritin level were tested.Results: The mean age of the patients was 10.3 years (47.6% above 10years and 52.4% under 10years old). This study demonstrated that fourteen patients (21%) had hypothyroidism (1.5%overt low T4 and high TSH), which 7.6% was sub clinical (normal T4and high TSH and 12.3% secondary hypothyroidism) in other study prevalence hypothyroidism was 2.2%-16.5%.There was no correlation between hypothyroidism and serum ferritin level (P-value= 0.38).Conclusion: The high rate of hypothyroidism indicates the importance of regular follow up of thalassemia patients to prevent complications.

Background: Glucantime is the first line agent for treatment of cutaneous leishmaniasis (CL). It has adverse effects on blood elements. This research has been done to evaluate the blood complications of this medicine in patients with cutaneous leishmaniasis.Methods: This clinical trial was done at Nikpour clinic, Yazd, Iran. Blood samples were collected from patients diagnosed with CL before treatment and after receiving 20 mg/kg intramuscular glucantime. Injection every day for 20 days. Full cell blood count was done for all patients. Statistical analysis of data was achieved by paired-T-test using SPSS software (version 13). Results: The blood tests results showed glucantime significantly decreased RBC, PLT, WBC (except monocytes), Hb and Hct (P<0.05). MCH and PMN decreased but not significantly. MCV, MCHC and eosinophil count increased but not significantly. No correlation was seen between laboratory test results and patients' age and sex.Conclusion: Glucantime affected the blood indices and it was suggested to use other alternating therapy. Future study with bigger sample size should be done for the more clear results.

Background: A vast variety of factors may cause recurrent pregnancy loss. Blood group incompatibility of parents could cause abortion. The examination of couples or twins, blood groups showed that the blood group incompatibility can affect adversely the outcome of pregnancy. Couples with blood group incompatibility are more involved in spontaneous miscarriage. Antigenes in two different blood groups could disorganize implantation. Aborted embryos with normal karyotype showed more frequently blood group incompatibility with their mother. Abnormal newborn and stillbirth were observed more frequent in couples with incompatible blood groups than without. Methods: This study investigates relationship between blood groups incompatibility and recurrent miscarriage in couples who were referred to genetic counseling clinic of Yazd Research & Clinical Center for Infertility. The blood group of 100 couples with recurrent miscarriage was evaluated using slide test method. Their abortions were unknown after possible evaluation. One hundred and twelve fertile couples entered to this study as control group, which have at least two normal children without any abortion. Results: The results showed blood group incompatibility was more frequent in couples with recurrent abortion than fertile couples. Conclusion: Blood group incompatibility of parents could causes antigen-antibody interaction between mother and fetus, which ended with abortion. In previous study it was believed that blood group incompatibility cases fetal anemia and stillbirth.

Arterial ischemic stroke defines as a new focal neurologic deficit that lasted 24 hours or longer. Stroke is relatively rare in children and incidence of cerebrovascular disease is 1 per 4000 in neonates and 1 per 7000 to 1 per 70000 in older children (1 month to 18 years). Protein S deficiency is one of the causes of the stroke in children. Major manifestations of protein S deficiency are deep venous thrombosis, superficial thrombophlebitis, and pulmonary emboli. The pathogenesis of vascular occlusion in patients with protein S deficiency is unknown. The prevalence of protein S deficiency is between 1% to 7% for first episode of deep venous thrombosis. We present a case of the 6-year-old boy with a history of tonic clonic sizure within a period of 3 years. He subsequently developed acute aphasia and right hemiparesis. Brain magnetic resonance imaging revealed lacunar infarction. Laboratory finding showed that low level of total protein S. The patient was treated with intravenous heparin and patient completely improved.There are no evidence based strategies for the treatment of children with prothrombotic abnormality (because of the lack of research on this subject). However this patient completely improved and discharged with oral anticoagulant.