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Background: Idiopathic thrombocytopenic purpura (ITP) is a rare and autoimmune disorder determined by an abnormal reduction in the number of platelets. The current study aims to evaluate the oxidative stress status of children with ITP in two treatment methods using methylprednisolone and methylprednisolone with intravenous immunoglobulin (IVIG). Materials and Methods: This retrospective study was conducted on 60 children with ITP who referred to Baghaei Hospital in Ahvaz in 2021. All the ITP children were equally divided into two groups, 30 receiving methylprednisolone and 30 receiving methylprednisolone and IVIG. The sampling of the patients’ blood was done in two stages before and after the start of treatment. Then, malondialdehyde (MDA), superoxide dismutase (SOD), total antioxidant status (TAS), total oxidative status (TOS), catalase (CAT) and glutathione were measured according to the instructions in the commercial kit. The analyses were performed using SPSS software version 23. P value < 0.05 was significant. Results: The number of platelets after treatment in methylprednisolone and methylprednisolone+ IVIG groups was 133.44 ± 18.93 and 158.76 ± 34.76 (×103/μL), respectively. Itas significantly increased compared to that before the treatment (P = 0.04). The amount of TAC in the group receiving methylprednisolone + IVIG and the methylprednisolone group was 1.64 ± 0.18 and 1.26 ± 0.53 nm, respectively; there was a remarkable difference between the two groups (P = 0.001). Also, SOD, CAT and glutathione in the methylprednisolone + IVIG group were remarkably higher than those in the methylprednisolone group (P < 0.05). Finally, the levels of TOS were lower in the methylprednisolone + IVIG group (19.74 ± 9.93 μmol) than in the methylprednisolone group (26.65 ± 10.64 μmol) (P = 0.01). Conclusion: A combination of IVIG and methylprednisolone was found to have a greater effect on improving antioxidant status and decreasing the oxidative stress indices of ITP children. [ABSTRACT FROM AUTHOR]

Background: Despite the low quality of life (QoL) of children with leukaemia, there is a lack of well-designed and culture-oriented care programmes to improve it. The Partnership Care Model (PCM), which was developed based on the Iranian culture, seems to be effective in improving the QoL in children living with chronic diseases. Aim: This study aimed to evaluate the effect of a care programme based on the PCM on the QoL of children with leukaemia. Methods: This controlled clinical trial encompassed 60 school-age children with leukaemia and their mothers. In the experimental group (n=30), a 2-month care programme was implemented based on the PCM, including the four stages of motivation, preparation, involvement and evaluation. Only standard care was provided to the control group (n=30). Children's QoL was assessed using the Paediatric Quality of Life Inventory 3.0 Cancer Module in the pre-test phase and 2 months after the intervention. Findings: The total score of QoL and all its subscales were significantly higher in the experimental group than the control group in the post-test phase (p<0.001). Moreover, a significant difference was observed between pre-test and post-test scores in all domains in the experimental group (p<0.001); however, no significant difference was noticed between the scores in the control group. Conclusion: The partnership care programme seems to improve the QoL in children with leukaemia. Future investigations are recommended to shed further light on the findings of this study. [ABSTRACT FROM AUTHOR]

Background: Genetic risk factors are implicated in the etiology and pathogenesis of immune thrombocytopenic purpura (ITP). Platelet endothelial aggregation receptor 1 (PEAR1) plays an important role in regulating megakaryopoiesis and thrombopoiesis. rs12041331 and rs12566888 single-nucleotide polymorphisms of PEAR1 are associated with megakaryocyte differentiation and platelet function. Materials and Methods: To conduct this study, 68 peripheral blood samples of patients with ITP (56 acute and 12 chronic) were collected. The amplification refractory mutation system-polymerase chain reaction (ARMS-PCR) was used to detection of rs12041331 and rs12566888 PEAR1 polymorphisms. Results: Statistically significant differences were not seen between rs12041331 and rs12566888 genotypes in acute and chronic groups (P = 0.778, P = 0.844). The frequency of rs12041331 AG/AA genotypes and the rs12566888 GT genotype was more in acute ITP patients; on the other hand, the rs12566888 TT genotype was more in the chronic group. The highest platelet counts and platelet distribution width (PDW) were related to the rs12041331 AG allele. GT and TT of rs12566888 had more PDW and platelet count, respectively. Mean platelet volume values between alleles of both the polymorphisms were constant and did not differ much. In general, no statistically significant differences were observed between genotypes of polymorphisms and platelet parameters. Conclusions: There was no association between rs12041331 and rs12566888 with platelet parameters in ITP patients and the severity of this disease. Further investigation with a larger size is recommended. [ABSTRACT FROM AUTHOR]

Key Clinical Message: The accurate diagnosis of Kikuchi‐Fujimoto disease can protect children from unnecessary diagnostic procedures and treatments. Also, the co‐occurrence of rare diseases with other diseases can improve or worsen the symptoms of the patients. [ABSTRACT FROM AUTHOR]

Context: Immune thrombocytopenia (ITP) is an autoimmune disease that is caused by the dysregulation of immune system, in which the circulating platelets (Plt) are destroyed by reticuloendothelial system, leading to hemorrhagic manifestations in patients. Aims: Recent studies have indicated that polymorphisms can contribute to ITP susceptibility and outcome. Given the importance of follicular helper T (Tfh) cells in ITP, we evaluated polymorphism in Inducible T-cell Costimulator (ICOS) as a Tfh surface marker among ITP patients to find a likely prognostic factor. Subjects and Methods: We recruited 54 ITP patients and 46 persons with no history of thrombocytopenia to conduct this case–control study. In addition to routine laboratory parameters, three polymorphisms, namely rs10932036, rs4404254, and rs10932037 of ICOS gene, were assessed by polymerase chain reaction. Statistical Analysis: Mann–Whitney, Kruskal–Wallis, and Chi-square tests were employed to compare and evaluate the data, and P < 0.05 indicated a statistically significant association. Results: The findings of our study showed that allele and genotype frequencies of all three polymorphisms in question were similar between case and control with no significant difference. However, our assessment indicated higher mean Plt counts in RS4404254 CC genotype than other genotypes under investigation. Conclusions: It seems that rs10932037, rs4404254, and rs10932036 polymorphisms of ICOS gene are not involved in susceptibility to ITP. Nevertheless, we found that those carrying RS4404254CC polymorphism have better prognosis due to higher Plt counts both in acute and chronic ITP patients. [ABSTRACT FROM AUTHOR]

Background Immune thrombocytopenia (ITP) is an autoimmune disease characterized by symptoms of thrombocytopenia and bleeding due to production of autoantibodies against platelets. Recently, the occurrence of polymorphisms has been identified as one of the main causes of disease onset. Methods To conduct this study, we recruited 140 patients and control individuals with no history of platelet loss. After collection of specimens, the prevalence of interferon-γ polymorphism was evaluated using the allele-specific oligonucleotide–polymerase chain reaction (ASO-PCR) technique and confirmed by sequencing techniques. Results The results showed that the frequency of the AA genotype was higher in the control group, compared with patients with ITP; however, in the acute and chronic groups, the frequency of the AT genotype was higher than that of the AA genotype. We also discovered that there was no significant correlation between platelet counts before and after treatment, nor in its related parameters with interferon (IFN)–γ polymorphism. Conclusion rs2430561 does not seem to have any role in ITP pathogenesis and treatment response. [ABSTRACT FROM AUTHOR]

Background: Cytogenetic abnormalities in leukemia cells have strong prognostic values for different clinical subgroups, clinical features, and therapeutic outcomes. Patients and Methods: This study was conducted on 100 children with acute lymphocytic leukemia referred to Ahvaz Shafa Hospital during 2012–2017. The patients were diagnosed by a specialist through examination of morphology and flow cytometry, testing bone marrow specimen on the 7th day of treatment, and a karyotype and cytogenetic test are performed. Results: There was no relationship between the t(11q23) and gender nor age of children. Besides, the mean white blood cell (WBC) counts in patients who were negative for 11q23 and those positive revealed a statistically significant relationship between WBC count and 11q23 (P = 0.022). Conclusion: A significant association between the 11q23 translocation and primary response to chemotherapy is existed. Diagnostic accuracy of these tests for detecting t(11q23) is generally high, as well as sensitivity and specificity are optimal for all anomalies. [ABSTRACT FROM AUTHOR]

Immune thrombocytopenic purpura (ITP) is an autoimmune bleeding disorder associated with platelet destruction. Abnormalities in frequency and function of different immune cells can play a crucial role in this disease. The aim of this study was to evaluate the prognostic value of CD markers’ expressions by immune cells in ITP. Peripheral blood samples were collected from 25 ITP patients before and after treatment. The expression of CD markers was evaluated by flow cytometry technique. The expression of CD38 and CD56 was significantly lower before treatment than after it (p = 0.025 and p = 0.036, respectively). Furthermore, a positive correlation was found between CD38 expression with platelet count before (r = 0.496, p = 0.012) and after treatment (r = 0.404, p = 0.045). No significant relationship was found between this marker and platelet count while CD4 expression was higher before treatment than after it (p = 0.002). In conclusion, CD38 may have independent prognostic value in ITP and we suggest that it can be a prognostic marker for this disease. [ABSTRACT FROM AUTHOR]

Chronic myeloid leukemia (CML) is a hematopoietic stem cell malignancy characterized by the expression of the BCR-ABL1 fusion gene with different chimeric transcripts. Despite the crucial impact of constitutively active tyrosine kinase in CML pathogenesis, aberrant DNA methylation of certain genes plays an important role in disease progression and the development of drug resistance. This article reviews recent findings relevant to the effect of DNA methylation pattern of regulatory genes on various cellular activities such as cell proliferation and survival, as well as cell-signaling molecules in CML. These data might contribute to defining the role of aberrant DNA methylation in disease initiation and progression. However, further studies are needed on the validation of specific aberrant methylation markers regarding the prognosis and prediction of response among the CML patients. [ABSTRACT FROM AUTHOR]

Immune Thrombocytopenic Purpura ( ITP) is a common autoimmune bleeding disorder characterized by a reduction in peripheral blood platelet counts. In this disease, autoantibodies (Auto-Abs) are produced against platelet GPIIb/ GPIIIa by B cells, which require interaction with T cells. In this review, the importance of B and T lymphocytes in ITP prognosis has been studied. Relevant literature was identified by a PubMed search (1990-2016) of English-language papers using the terms B and T lymphocyte, platelet, CD markers and immune thrombocytopenic purpura. T and B lymphocytes are the main immune cells in the body. Defective function causes disrupted balance of different subgroups of lymphocytes, and abnormal expression of surface markers of these cells results in self-tolerance dysfunction, as well as induction of Auto-Abs against platelet glycoproteins ( PG). Given the role of B and T cells in production of autoantibodies against PG, it can be stated that the detection of changes in CD markers' expression in these cells can be a good approach for assessing prognosis in ITP patients. [ABSTRACT FROM AUTHOR]

Wilms' tumor is common in children and is caused by Wilms' tumor gene 1 (WT1). The WT1 mutation has been reported in a variety of hematologic malignancies. Changing expression of miRNA molecules has also been shown to play a role in the development of Wilms' tumor. Considering the fact that WT1 can be used as a clinical biomarker in leukemia cases, it can be a basis for immunotherapy of leukemia. WT1 is a gene that can be used as a prognostic biomarker for minimal residual disease, as well as the detection of relapse for clinical remission in leukemia. Furthermore, it can be considered as a predictive biomarker for the treatment of leukemic patients after allogeneic transplantation. This study aimed to review WT1 expression in leukemia, its involvement in miRNAs expression, as well as its importance in prognosis and treatment of leukemia. [ABSTRACT FROM AUTHOR]

The haplotypes of β-globin gene cluster in patients with sickle cell anemia are associated with different clinical manifestations and fetal hemoglobin (HbF) levels. In addition, SNP in some genes within the β-globin gene cluster (HBBP1) and outside it (HMOX1) has been recently reported to be related to HbF levels. In this study, rs2071348 and rs2071746 polymorphisms in HBBP1 and heme oxygenase 1 (HMOX1) genes associated with different haplotypes and HbF levels in patients with sickle cell anemia were evaluated in comparison with healthy subjects. In this case-control study, 150 patients with sickle cell anemia (90 homozygous and 60 sickle-cell trait) were evaluated and compared with 50 healthy individuals referred to Shafa tertiary hospital in Ahvaz city of Khuzestan Province, Iran. HbF level was measured by alkali and acid electrophoresis. PCR-RFLP and ARMS-PCR methods were used to determine β-globin gene haplotypes and HBBP1 gene polymorphism, respectively. Arab-Indian was the most common genotype found in both homozygous and sickle-cell trait patients with a respective prevalence of 55.5 and 50% followed by homozygous Benin with 11.1 and 18.3% prevalence, respectively. The highest and lowest level of HbF were observed in Arab-Indian (24.1 ± 6.35) and Benin/Benin haplotypes (7.63 ± 2.29), respectively. A significant relationship was found between HbF level with rs2071746 A>T polymorphism of HMOX1 gene ( P < 0.019) as well as with rs2071348 polymorphism of HBBP1 gene ( P < 0.034). However, there was no significant relationship between haplotypes with gene polymorphisms. Arab-Indian was the most common haplotype in this region. The HbF level was significantly higher in patients with Arab-Indian haplotype, patients with C genotype in rs2071348 polymorphism locus of HBBP1 gene, and patients with T genotype in rs2071746 polymorphism locus of HMOX gene. [ABSTRACT FROM AUTHOR]

Acute leukemias of ambiguous lineage (ALAL) form a group of leukemias presenting the features of both acute lymphoid leukemia (ALL) and acute myeloid leukemia (AML). Based on Immunologic Classification of Leukemia (EGIL), these leukemias are recognized as biphenotype acute leukemia (BAL). According to WHO classification and based on immunophenotypic characteristics, ALAL is further categorized to acute undetermined leukemia (AUL), mixed phenotype acute leukemia (MPAL), and other ambiguous lineage leukemias. Immunophenotype and cytogenetic aspects are important in recognition of these leukemias; however, there is limited data about these leukemias. This has resulted in mistreatment of patients. Thanks to the recent advances and introducing the role of microRNAs in regulation of gene expression, a new avenue has been opened in the field of classification and pathogenesis of ambiguous lineage leukemias. MiRNA-based classification can help define myeloid or lymphoid origin of ambiguous lineage leukemias. Taking immunophenotype, cytogenetic, and miRNA profiles into account collectively, a better recognition of the disease is achieved, which results in accurate diagnosis and effective treatment of patients. In this review, we have discussed ambiguous lineage leukemias with regard to microRNA expression, immunophenotype, and cytogenetic aspects. [ABSTRACT FROM AUTHOR]

Acute lymphoblastic leukemia is a hematological malignancy of lymphoid progenitor cells associated with excessive proliferation of lymphocytes, and lymphocyte markers can be used for the diagnosis and assessment of disease. Cell surface receptors, including T cell receptor and immunoglobulin (on T and B cells, respectively), are among the most important lymphocyte markers. Gene segment variation, i.e., involvement of several genes in the expression of each receptor, is effective on extensive rearrangement of cell surface receptors. These receptors can be observed in both T-acute lymphoblastic leukemia (ALL) and precursor B-ALL cells. In this study, we have discussed T cell receptor (TCR) gene rearrangements in ALL. As leukemia cell proliferation originates from a unique clone, clone-specific rearrangement can be very helpful in diagnosis and detection of remaining malignant cells from among normal cells for minimal residual disease. On the other hand, each of the TCR genes is associated with translocation in ALL types, and the result of gene rearrangements can be used as prognosis markers. [ABSTRACT FROM AUTHOR]

Background: Acute lymphoblastic leukemia (ALL) is a malignant disorder of lymphoid progenitor cells that affects both children and adults. B-lineage acute lymphoblastic leukemia (B-ALL) that derived from primary B cell precursors is acommonsubtype of ALL. PAX5 is a member of PAX gene family. This gene is located at 9p13.2, encoding the B-cell lineage specific activator protein (BSAP). BSAP is an essential regulator of B lymphocytes identity and function which plays an important role in part of B cell specific genes. Objectives: The aim of this study is to screen probable variants in flanking regions of introns 1 and 2 near the exons 1, 2 and 3 of PAX5 gene among B-ALL patients from Khuzestan province. Patients and Methods: In this descriptive study, blood samples were collected from 50 patients with clinical symptoms of B-ALL in Khuzestan province. In order to identify the probable variants in introns 1 and 2 near the exons 1, 2 and 3 of PAX5 gene, flanking regions of introns amplified by PCR and the products were sequenced for any probable change. Results: Two variants in nine patients were identified including IVS2-43T > C and IVS2 + 11T > G. IVS2-43T > C variant was found as a heterozygous form in one patient and IVS2 + 11T > G was found as a homozygous variant in 8 patients with B-ALL. Conclusions: The overall frequency of variants in intron 2 of PAX5 gene was 18%. IVS2 + 11T > G variant of PAX5 gene probably do not associated with B-ALL risk in the population. [ABSTRACT FROM AUTHOR]

MicroRNAs (miRNAs) play an essential role in the development and progression of acute lymphoblastic leukemia (ALL), and could serve as disease biomarkers and therapeutic targets. The function of miR-146a in lymphoid differentiation has been here with discussed. However, the role of this miRNA in the outcome of ALL is not well understood. Peripheral blood of 48 patients with ALL and 20 age- and sex-matched healthy control subjects was used to accurately evaluate the expression of miR-146a by stem-loop Real time PCR. No statistically significant difference was found between patients and controls in total miR-146a expression. The expression of miR-146a was high (18.75%), low (27.08%) and not different (54.17%) in ALL patients. Our analysis indicated no association between the expression of miR-146a and any prognostic factors such as WBC/PLT counts, Hb, fusion genes (P190 and some translocations) with ALL type. This study revealed that miR-146a cannot be an independent factor for predicting the outcome of ALL patients.We suggest a multi-parameter analysis including miRNAs, transcription factors and critical genes to achieve a precise clinical panel for prognostic values. [ABSTRACT FROM AUTHOR]

β-Thalassemia is a genetic disorder with a continuum of mild to severe clinical manifestations and requirement of transfusion at different stages of life. The cause(s) of this variety is not clear but genetic alterations could be a potential factor. In this review, the correlation between polymorphisms and different clinical manifestations, including the need for transfusion, was investigated. Relevant articles published in pubmed database from 1982 onwards were studied and compiled. The articles all contained the keywords β-thalassemia, genetic modifiers, and mutations. Certain polymorphisms and mutations could dictate the severity of symptoms as well as their onset. A significant number of the mentioned genetic alterations appear in beta- globin gene cluster and affect gamma chain. Therefore, hemoglobin F production rate is increased and can affect thalassemia symptoms and can relieve β-thalassemia symptoms. A number of polymorphisms in catalase and glutathione S transferase genes have also been shown to modify the severity of disease and response to treatment. Knowledge of these mutations and polymorphisms can provide an insight into the prognosis for individual patients, especially in young ages or before birth to take proper measures in advance and eventually ameliorate the symptoms in the long run. [ABSTRACT FROM AUTHOR]

Chemokines secreted from different cellular components of bone marrow (BM) play an important role in the formation of the BM niche system. The hematopoietic stem cell (HSC) pool located in specialized anatomical sites within the BM is subjected to a complex network of chemokines, such that the produced chemokines affect the fate of these cells. Expression of different chemokine receptors on leukemic stem cells (LSCs) uncovers the critical role of chemokines in the maintenance, survival and fate of these cells in the leukemic niche. As a pre-metastatic niche rich in a variety of chemokines, the BM niche is turned into a locus of tumor cell development and division. The chemokine receptors expressed on the surface of metastatic cells lead to their metastasis and homing to the BM niche. Knowledge of chemokines and their receptors leads to the production of various therapeutic antagonists at chemokine receptors expressed on leukemic and tumor cells, enabling interference with chemokine function as a therapeutic tool. New findings suggest that miRNAs, with their specific inhibitory function, affect the ability of producing and expressing chemokines and chemokine receptors. This review focuses on the emerging role of chemokines and their receptors in normal and pathologic conditions of the BM niche, and also discusses the new therapeutic methods with this background. [ABSTRACT FROM AUTHOR]

Objective Bone marrow (BM) niche is a three-dimensional structure composed of a series of cells and it is one of the most controversial topics in hematological malignancies, leukemia, and even metastasis. Here, we review the relationship between Notch signaling and different fates of stem cells and other BM niche cells. Methods Relevant English-language literature were searched and retrieved from PubMed (2000–2013) using the terms Notch signaling, BM niche, and microRNAs (miRNAs). Discussion Notch signaling pathway is a signaling system involved in cellular processes such as proliferation, differentiation, and apoptosis. The notch signaling pathway components are associated with interaction between leukemic, metastatic, and normal cells and their microenvironment. miRNAs play an important role in expression and regulation of signaling molecules. It is necessary to evaluate the relationship between aberrant miRNA expression and notch signaling such as miR-128 and miR-30 in glioma and angiogenesis with notch signaling, respectively. Conclusions Characterizing malignant cells and future studies focus on better understanding the variety of cancers and apoptosis with activated Notch signaling pathway, may remain promising this signaling system as a safe and effective therapeutic target. [ABSTRACT FROM AUTHOR]

Introduction: Acute leukemia is the most common malignancy in children and acute lymphoblastic leukemia (ALL) accounts for 75% of acute leukemia cases. New treatment protocols have resulted incomplete remission rates up to nearly 100% in children with acute lymphoblastic leukemia. Today, one of the most important prognostic factors in acute lymphoblastic leukemia is intensity of the treatment. Risk stratification is accomplished based on clinical, morphological, immune-phenotypic and cytogenetic findings. The aim of this study was to determine some prognostic factors in children with acute lymphoblastic leukemia. Methods: In this retrospective study information about age at onset of acute leukemia, sex, initial white blood cell count, FAB-subtype, immunophenotype, and clinical course of newly diagnosed acute lymphoblastic leukemia were extracted from medical records of children admitted to pediatric oncology department of ShafaHospital between- 2011and2012. Results: There were 21 male patients (51.2%) and 20 were female patients (48.8%). The mean age was 4.2 ± 6.34 years, and 24 patients (58.5%) had Arab origins, while 17 patients (41.5%) were of non-Arab ethnicity. Age distribution showed higher incidence of ALL in younger children: 1-4 years 47.5%, 5-9 years 27.5% and 25% in patients >10 years.L2 subtype was more common in our patients 51.2% while L1 subtype was reported 46.3%.Only one patient was reported to be L3 subtype (2.4%), yet we did not detect any significant relation between different age groups and trend for incidence for specific subtype. The number of white blood cell (WBC) at the time of admission was reported as: less than 10,000 cells/cm in 30%, between11-50,000 in 37.5% and >50,000 in 32.5% patients. Organ involvement was present in 47.5%, and central nervous involvement, (proved by positive malignant cells in CSF fluid) was detected in 4.9% of our patients. In our study, HLA-DR was 62.5% in ALL patients and CD 20 and CD19 was the most common marker in these patients. In our work the most common markers in L1, which was found in 19 patients were reported CD 19, CD33, CD22,CD35, CD20 and CD9. Also the percentage of markers in L2 subtype had a similarity to L1 group. Conclusion: Conclusion: In this study, FAB-subtype L1 was less than previous studies, while FAB-subtype L2 and pre-B cell immunophenotype was more common than previous studies. Other results were the same as reported in older studies. [ABSTRACT FROM AUTHOR]

Background: Immune thrombocytopenic purpura (ITP) is an autoimmune disease that can cause bleeding disorders in patients, and presents in acute and chronic forms. The acute form is frequently seen in children, but the chronic form mainly inflicts adults. There are differences and similarities in clinical and laboratory findings of the disease between children and adults. We study these differences and similarities in these two groups of patients with ITP. Methods: In this study, we retrospectively evaluated the clinical and laboratory data of 323 ITP cases within three years. None of our patients had a history of thrombocytopenia. Patients were classified into two groups of children (3 months to 16 years of age) and adults (⩾ 16 years). Data analysis was conducted using SPSS software, and the analysis results were compared between the two age groups. Results: Overall, the disease prevalence was higher in women than men, but the prevalence of childhood ITP was higher in males than females. The prevalence of initial symptoms including petechiae, purpura and ecchymosis was 60.5% and 61%, respectively in all patients, but severe bleeding rarely occurred in patients (28.8%). 30.5% of patients had a history of infection before developing ITP, and the children had a higher frequency of infection (80.8%). Before treatment, the mean platelet count in adults and children was 33000/μL and 35000/μL, respectively. Conclusion: Comparison of data in children and adults with ITP indicated similarities and differences in clinical and laboratory findings between the two groups with differences in prevalence, bleeding symptoms, initial platelet count and infection history. [ABSTRACT FROM AUTHOR]

T cell acute lymphoblastic leukemia (ALL) is an invasive disease with a higher incidence in children and adolescents. In terms of Immunophenotype, T-ALL is positive for CD2, CD7, CD34 and HLA-DR, and the level of these markers is increased with increasing age. In addition, the myeloid markers (CD13, CD33) are sometimes expressed in T-ALL. In this study, we introduce a rare case of a 28-year-old woman with T-ALL with aberrant expression of myeloid markers (CD13), without lymphadenopathy and with 94% blasts in bone marrow specimens. The patient has the rare karyotype of 46,XX del(7)(q11.2q22). The presence of del7 is a rare phenomenon in T-ALL. [ABSTRACT FROM AUTHOR]

Objective: The use of fetal hemoglobin (HbF) inducer drugs is considered as a novel approach in treatment of β-hemoglobinopathies, especially β- thalassemia and sickle cell disease. HbF inducers including hydroxyurea, histone deacetylase (HDAC) inhibitor agents such as sodium butyrate, azacitidine, decitabine and new immunomodulator drugs like pomalidomide, lenalidomide and thalidomide can reduce α-globin chain production in erythroid progenitors and improve α: β chain imbalance, the most crucial complication of β-thalassemia. Materials and Methods: In this article, we reviewed more than 40 articles published from 1979 to 2012 in the field of fetal hemoglobin augmentation. Results: Recent studies suggest the synergistic effect of drug combinations in efficient induction of fetal hemoglobin and gene over-expression. Conclusion: It seems that drugs which act with different molecular and epigenetic mechanisms have proper synergistic effects in fetal hemoglobin induction and gene over-expression. [ABSTRACT FROM AUTHOR]

Background: Hemoglobinopathy and thalassemia are prevalent genetic disorders throughout the world. Beta thalassemia is one of these disorders with high prevalence in Iran, especially in Khuzestan province. In this study, the rate of different mutations in β-globin gene for prenatal diagnosis in fetal samples was evaluated. Materials and methods: In this experimental pilot study, 316 fetal samples (chorionic villus or amniotic fluid) suspicious to hemoglobin disorders were enrolled. Afterwards, DNA was extracted and PCR and DNA sequencing were used for evaluation of different mutations in β-globin gene. Results: Amongst 316 samples evaluated for prenatal diagnosis, 180 cases (56.8%) were carrying at least one mutated gene of β-thalassemia. In addition, results showed that CD 36-37 (- T) and IVS II-1 (G>A) polymorphisms are the most prevalent polymorphisms of β-thalassemia in Ahvaz city with 13.9% and 10.1% rates, respectively. Conclusion: Using molecular tests for prenatal diagnosis is considered an efficient approach for reducing the birth of children with hemoglobinopathy and identification of prevalent mutations in each region. [ABSTRACT FROM AUTHOR]

Objectives: To investigate the frequency of causes of anaemia among adolescent school girls in Ahvaz in 2009. Methodology: A cross- sectional study was conducted on 208 Adolescent girls aged 15-19 years from high schools in Ahvaz. Blood samples were taken from all patients and were sent to laboratory for determination of complete blood count (CBC) test, serum ferritin (SF), hemoglobin electrophoresis (Hb F, Hb A2) was done for all of the anaemic cases. Iron deficiency anaemia was defined as a situation where Hb is less than 12 g/dl and ferritin is less than 12 ng/ ml. Beta-thalassemia were identified by high performance liquid chromatography (HPLC) analysis of hemoglobin and Hb A2>3.4. Result: Of the 208 patients, 37 cases (17.8%) were anemic and Iron deficiency anaemia was found in only 11 from 208 children (5.3%), minor beta thalassemia was found in only five of 208 children (2.4%), mixed beta thalassemia and iron deficiency anaemia was found in four of 208 (1.9%) and other patient with anaemia 19 patient (9.1%) minor alpha thalassemia was considered. anybody of students were macrocytic anaemia. Conclusion: The results suggest that thalassemia and iron deficiency anaemia may be major contributing factors to the occurrence of anaemia in this area among schoolgirl population. [ABSTRACT FROM AUTHOR]

Neuroblastoma is the most common extracranial solid tumor in children. The cooccurrence of neuroblastoma and acute lymphoblastic leukemia is rare. We report a rare case of advanced-stage neuroblastoma in a 3.5-year-old girl that was accompanied by acute lymphoblastic leukemia following treatment. Her leukemia may have been due to chemotherapy (secondary leukemia) or may have been de novo. [ABSTRACT FROM AUTHOR]

Neuroblastoma is the most common extracranial solid tumor in children. The cooccurrence of neuroblastoma and acute lymphoblastic leukemia is rare. We report a rare case of advanced-stage neuroblastoma in a 3.5-year-old girl that was accompanied by acute lymphoblastic leukemia following treatment. Her leukemia may have been due to chemotherapy (secondary leukemia) or may have been de novo. [ABSTRACT FROM AUTHOR]

Essential thrombocythemia is a rare myeloproliferative disorder in pediatrics. This myeloproliferative disorder is charactherized by thrombocytosis and hyperplasia of megakaryocytes in the bone marrow. Other cell lines are not involved.JAK2V617Fmutations has been identified in approximately half the patients with this disorder. We describe a 12-year-old boy with essential throbocythemia. The patient had a persistent thrombocytosis over 600×109 /L and the time of diagnosis, his platelet count ranged between 900×109and 2150×109/L. Megakaryocytes in the bone marrow were increased in number. The chromosomal analysis was normal and bcr/abl rearrangement was negative. He remained asymptomatic throughout the follow-up period. [ABSTRACT FROM AUTHOR]

Polyglandular autoimmune syndrome type I (PGA I) is a rare disease. Its hallmarks are chronic mucocutaneous candidasis, hypoparathyroidism and adrenal insufficiency. Immune thrombocytopenic purpura (ITP) is one of the most common autoimmune disease in children. Association of PGA I with ITP was not found in some previous studies, but, we report a child with PGA I and ITP. [ABSTRACT FROM AUTHOR]