International Journal of Hematology-Oncology and Stem Cell Research
Volume:5 Issue: 4, Oct 2011

Post-remission treatment in patients with acute myeloid leukemia is still controversial. One potent choice for patients with no donor available is autologous stem cell transplantation. The median follow-up period was 18 months and the median age was 26 years old. In the review of 116 recently diagnosed AML patients (except AML- M3) who underwent autologous stem cell transplantation, 72.4% of patients remained alive and 27.6% relapsed. Relapse was the most common causes of death in patients. The one- year OS and DFS of patients was 73.8% and 59.8%, respectively. There was no statistically significant difference between age, sexuality and waiting time from diagnosis to transplantation. For more comprehensive results, longer follow- up is required.

Hemoglobin F (HbF, α2γ2) is a major contributor to the clinical heterogeneity and ameliorating agent observed in patients with the β-globin disorders including β-thalassemia and sickle cell disease (SCD). During fetal life, HbF is the major hemoglobin but is largely substituted by adult hemoglobin (HbA, α2β2) following a globin expression switch after birth. Increased γ-globin expression can reduce the clinical severity of β-thalassemia and SCD. Therefore, increase in HbF production has served as a longstanding goal. The progression of target-based therapeutics has been confused by limited comprehension of molecular mechanisms of gamma-globin gene expression. However, recent discoveries of regulators of HbF level represent a major development and provide new opportunities in employing novel rational therapeutic strategies. In this review, molecular mechanisms of hemoglobin F induction will be discussed.

Myeloproliferative neoplasms (MPNs) are a group of clonal malignant hematologic disease, where the main and common members are; polycythemia vera (PV), primary myelofibrosis (PMF), and essential thrombocytosis (ET). These group of diseases are able to be transformed into each other. This cross sectional study conducted the evaluation of JAK2V161F mutation in DNA in peripheral blood of 91 patients with known or suspected diagnosis of MPNs by Real time PCR method and survey of peripheral blood smear. . Prevalence of JAK2V617F mutation between patients was %58.2 and PV had most common prevalence ratio between other groups. While mean age of patients was 50/9 yr (for 39.6% male and 60.4% female), three patients (equal of %3.3) were atypical presentation and 2 patients die due to malignant transformation. There were significant differences in age, WBC and PLT (in PV) with prevalence of JAK2V617F mutation. These differences were not significant in other group. Current study showed a high rate of association between JAK2V617F mutation in patients with PV, ET, PMF in Iranian patients. Therefore, Peripheral blood mutation screening for JAK2 V617F can be incorporated into the initial evaluation of patients suspected to have chronic myeloproliferative neoplasm and used of this test for determining of association between JAK2V617F mutation, treatment of patients with blood indexes and patients of prognosis.

Transfusion-transmitted infections (TTI) continue to be a major challenge for Blood transfusion organizations in the world and multi- transfused patients (MTPs) are at higher risk of infection. HTLV-1 is a retrovirus that easily transmitted via blood cell products. The aim of this study is determine the seroprevalence of HTLV-1 in major thalassemic patients from Kermanshah Province, western of Iran. A total of 116 serum samples from all major thalassemic patients that exist in Kermanshah providence and 1000 serum samples from healthy individuals as control group were tested for HTLV specific antibody by ELISA method. All of the ELISA positive samples were confirmed by Western Blotting analysis. From major thalassemic patients, 4 subjects (3.4%) had HTLV-I infection. Also, among 1000 control individuals, 5 subjects (0.5%) had HTLV-I infection. There wasn’t any HTLV-II in major thalassemic patients or control individuals. our results showed this infection exist in our region. These results indicated that screening procedure were not doing carefully. More studies are needed to clarify the reason of this unsuccessfully screening of this virus from donated blood.

Esophagus cancer is most common GI malignancies in Zahedan. This investigation was undertaken to define the epidemiology, and prognostic factors relevant to patients with Esophagus cancer in this region. We did a retrospective review of 175 patients with Esophagus cancer who presented to Ali Ebne Abitaleb Hospital and Khatam Alanbia Hospital in 2005-2010. Data analysis was done using the Kaplan-Meier method. The majority of the patients were male (70%) and 30% in female, and the mean age of them was 48.5 years. The vast majority were Iranian and from Baluch ethnicity group. 166 patients were Iranian population and in Balouchs ethnic group (118 cases) is more common than Sistani (49cases), and 9 patients were Afghan. Most of the cancer cases reported a history of Tobacco consumption (82 %) and the most common type of tobacco use was PAAN or Nass which is a combination of tobacco, lime, water, menthol and oil. Squamous Cell Carcinoma was the most commonly type of cancer (97%) in the region and adenocarcinoma was the second most common and the median survival time of patients was 13 months. Dysphagia and weigh lose were the most frequently encountered symptoms at the time of presentation. At the time of diagnosis early stage disease was found in 22%, locally advanced in 54% and metastatic in 24% of all cases. Any patients didn’t have a family history of Esophagus cancer. Clinical and epidemiological pattern of esophageal cancer in South-East of Iran is partially differed with other parts of the county as well as also other parts of the world which is important for both clinicians and health policy makers. Esophagus cancer is the most common GI malignancies in Zahedan. And this pattern is difference with other region in Iran, that, gastric cancer is most common GI malignancies according to previous of our study.

Severe acquired aplastic anemia (SAA) is a rare disease and matched related hematopoietic stem cell transplantation (HSCT) is the treatment of choice especially in pediatric patients. Immunosuppressive therapy is the alternative treatment in patients who do not have a donor. We retrospectively analyzed patients who received allogeneic HSCT at our institution. Between 1991 and 2011, 190 patients received allogeneic HSCT from HLA-matched donors (182 siblings and 8 other relatives). Median age was 20.5 years (range 1 to 50 years). The graft source was peripheral blood stem cells in majority of patients. Conditioning regimen consist with a myeloablative regimen containing cyclophosphamide with or without antithymocyte globulin. For graft-versus-host disease (GvHD) prophylaxis, we used cyclosporine with or without methotrexate at the standard doses. The median follow-up time was 30 months and 3 year overall survival and disease free survival was 82% and 75% respectively. The median time to neutrophil engraftment and median time to platelet engraftment was 12 day and 15 day respectively. Grade 3 and 4 of aGvHD occurred in 26 (23.7%) patients and chronic GvHD occurred in 46 (29.1%) of survived patients 100 days after HSCT. At time of report 82.1% of patients were alive with normal hematologic parameters. The engraftment failure rate was about 8%. The most common cause of death was GvHD. However an available treatment in SAA is immunosuppressive therapy, HSCT should be seriously considered as a therapeutic option particularly if a matched sibling donor is available. The outcome of allogeneic HSCT in patients with SAA at our center was consistent with the result of other previous studies.

There are only a few reports that exist regarding primary non-Hodgkin lymphoma of the genital tract. In this article, one case of primary B-cell type non-Hodgkin lymphoma (NHL) of the cervix is presented. Case history: A 45 year-old woman presented bloody vaginal discharge. Upon vaginal examination, the cervix was found to be enlarged. Cervical biopsy revealed B-cell type non-Hodgkin lymphoma. The patient was treated with CHOP chemotherapy. Systemic chemotherapy and radiation are recommended and effective treatments for genital tract B-cell type non-Hodgkin lymphoma.

Gastrointestinal stromal tumors (GISTs) are uncommon tumors of the gastrointestinal (GI) tract that occur predominantly in adults. These tumors originated from Cajal cells have positive mutations for KIT (CD117) or platelet-derived growth factor receptor (PDGFRA). GIST especially multiple GISTs are very rare in children and lacking mutations in KIT. We present a previously healthy 13 year- old girl with history of hematemesis and melena for two months which has caused severe anemia requiring ICU admission with final diagnosis of KIT positive multiple GISTs in stomach.