International Journal of Hematology-Oncology and Stem Cell Research
Volume:2 Issue: 3, Jul 2005

Reverse transcriptase-polymerase chain reaction (RT-PCR) assay is a useful tool for the detection of fusion transcript resulting from specific chromosomal translocation of the leukemia cells. A specific chromosomal abnormality, the Philadelphia chromosome (Ph), is present in 90% to 95% of CML patients.The aberration results from a reciprocal translocation between chromosome 9 and 22, creating a BCR-ABL fusion gene.There are two major forms of the BCR/ABL fusion gene, involving ABL exon 2, but including different exons of BCR gene. The transcripts b2a2 or b3a2 code for a p210 protein. Another fusion gene leads to the expression of an e1a2 transcript, which codes for a p190 pro-tein. Another, less common fusion genes are b3a3 or b2a3 (p203) and e19a2 (p230). The incidence of one or other rearrangement in chronic myeloid leukemia (CML) patients varies in different reported se-ries. In general, fusion transcripts are determined individually, a process which is labor intensive in or-der to detect all major fusion transcripts. This study was designed to determine the frequency of different fusion genes in 75 iranian patients with CML. peripheral blood samples were analyzed by multiplex reverse transcriptase poly-merase chain reaction (RT-PCR) from adult patients to detect all types of BCR-ABL transcripts of the t (9:22) and found that all cases were positive for some type of BCR/ABL rearrangement. Most of our patients showed b3a2 fusion gene (62%), while the remaining showed one of the transcripts of b2a2, b3a3, b2a3, e1a2 or coexpression of b3a2 and b2a2. The rate of coexpression of the b3a2 and b2a2 was 5%. In contrast to the other reports, we did not see any coexpression of p210/p190. This may reflect either the sensitivity of the detection techniques used or the possibility of genetic differences be-tween the populations studied. Coexpression may be due to alternative splicing or to phenotypic varia-tion, with clinical course different from classical CML.

Abstract: Breast cancer is the most common of all malignant neoplasms in women worldwide. This study aims to demonstrate certain biological, clinical and pathological characteristics of patients treated at the university hospital oncology unit. A descriptive study was conducted during a period of 2 years, from October 2003 through September of 2005 in Kermanshah, Iran. 555 patients were selected to participate, representing all the cases diagnosed and treated for breast cancer. Data was gathered according to questionnaires and pa-tients’ records. The mean age at which breast cancer was first diagnosed was 46.5±11.6 year of age with 89% of tumors being infiltrating intraductal carcinoma. The majority of the patient population had tumors stage II and grade II. Mean tumor size was 2.14±0.57 centimeters. 58% of the tumors were localized to the upper outer quadrant of the affected breast and 89% of the patients received modified radical mastectomies with almost a 92% two year survival. Highest prevalence of breast cancer was recorded in the 40-49 (mean 46) years of age group which compares favorably with studies done under similar circumstances. Tumor size, grade, stage, tumor marker analysis, metastasis and other disease characteristics portray patient population tendencies for breast cancer patients in Kermanshah, Iran.

Skull base meningiomas constitute a large proportion of the meningiomas, which are the most common benign brain tumors. The treatment of skull base meningiomas is specially challenging and controversial due to the proximity of these tumors to the vital brain structures. Radiosurgery is now emerging as an efficient alternative treatment modality which involves the ablation of tumor and the supplying blood vessels by a conformal dose of colliding gamma rays from 201 cobalt-60 sources. We here report the first 100 meningioma cases treated in Iran using Leksell Gamma knife model C system. Gamma knife treatment was performed by means of 18 grays at 50% isodose. Seventy percent of the total patients referred to the Gamma knife center were skull base men-ingiomas, 40% of patients were new cases of meningioma and the remainder had undergone one or more microsurgery procdeures. There was no mortrality associated with treatment. The most common complications were severe headache (10 patients) and peritumoral brain edema (9 patients). There was 95% tumor control rate as indicated by stable or reduced tumor volume. There was better clinical improvement in new cases relative to those with previous micro-surgery. Our study shows that gamma knife could be considered as primary or adjuvant therapy in all cases of meningioma specially the skull base meningiomas.

especially regarding roles for surgical resection.The aim of this study is evaluation of 5 years survival and methods of treatment of primary gastric lymphoma in a group of Iranian patients. The authors review the clinical features, staging, pathology, prognosis, and management of 30 patients with an emphasis on the role of chemotherapy, surgical resection and radiotherapy of 71 gastrointestinal lymphoma cases. A total of 30 patients (19 male and 11 female) with a mean age of 51 years and a range of 34 – 68 years were included in the study. The frequency of primary gastric lymphoma in our series was 42% of the total of primary gastrointestinal lymphoma. The overall survival rate was 47.8% at 5 years. Stag-ing usually was completed using noninvasive techniques. Patients with stage I or II disease were treated with Surgery (gastric resection) and chemotherapy showed improved Free Disease Survival (FDS) of 67% at 5 years. The five-year survival for stage I, II, III and IV patients were 87%, 61%, 25%, and 11% respectively, and the five-year survival for low grade and high grade were 91% and 56%, respec-tively. Stage III or IV and inoperable primary gastric lymphoma were treated with chemotherapy and radiotherapy showed improved Free Disease Survival (FDS) of 67% at five years. The five-year sur-vival for stage I, II, III, IV were 87%, 61%, 25% and 11% respectively, and the five year survival for low grade and high grade were 91% and 56% respectively. Early stage disease and high-grade Lymphoma have a better prognosis and patients who have complete surgical removal of primary tumor and chemotherapy.

Langerhans cell Histiocytosis (LCH) is a rare disease characterized by clonal prolifera-tion of Histiocytosis in different tissues. Permanent consequences (PC) described among subjects with Langerhans cell histiocytosis (LCH).In this study we report the prevalence of permanent sequel among long – term survivors of LCH in our center. We had 30 cases of LCH from 1989 – 2001 who came for at least 3 years after diagnosis. In-formation has been collected from their disease history, and on type and date of onset of any PC. The cumulative risks of developing a PC have been calculated from the date of LCH diagnosis using the Kaplan-Meier and non-parametric method. Among 30 patients 53.3% were female, 46.67% male, mean age at diagnosis 56.86%±7.79 months (range 7-156), median 42.5 months. 19 (63.33%) had single system (SS) and 11 (36.66%) mul-tisystem (MS). Mean age at SS 5.97 ±1.03 yr, mean age of MS 7.59±1.05 yr. Mean age at follow up 11.3 ± 0.9 yr, median 11.5 yr, range 4.16-22 yr. Mean duration of follow up 6.57 ± 0.76 yr, median 5 yr, and range 3-18 yr. Nine of 30 cases (30%) had at least 1PC; in SS (26.3 %) and in MS (36.7%). The most frequent PC was diabetes insipidus (DI) 16.7%, in SS 5.26%, in MS 36.36%, the difference is significant P<0.05.Orthopedic abnormalities 10% which was only in SS (15.79 %), growth retardation (GR) 13.34%. The prevalence of PC in our patients is low which could be due to a small sample and on the other hand as most of our patients had single system involvement, the exact prevalence of PC is not clear. Analysis of cumulative risk shows that some types of PC may become manifest many years from diagnosis and long term follow up is necessary for all patients.

Quantification of Epstein - Barr virus (EBV) in peripheral blood mononuclear cells (PBMNC) of allogenic bone marrow transplant (BMT) recipients is important because EBV-associated posttransplant Lymphoproliferative disease (PTLD) can occur after transplantation due to immunosup-pression therapy. To this end we chose Real-Time PCR using TaqMan probe. For the standard curve, we cloned BALF5 gene of EBV into a plasmid vector. After purification of the EBV-clone and calculation of plasmid copy number, the standard curve was constructed by using serial dilution of the plasmid clone. We were able to detect from 2 to 107 copies per 2×105 PBMNC with wide linear range. The mean EBV DNA copy number was 103.7 copies per 2×105 PBMNC. In this study, No patient of 35 BMT recipients (275 PBMNC samples) developed PTLD during five months follow up post transplant. EBV copy numbers in 22 samples (3 patients) out of 35 BMT recipients were higher than cut off value with symptoms like fever and pulomonary noddes (9%). The virus load in one patient in the last sample obtained was 72400 copies. We detected low levels of EBV DNA in 20 BMT patients (57/1%). Real-Time PCR is useful to measure virus load in PBMNC. Detection of EBV in PBMNC samples may be valuable predictive marker to prognosis PTLD. Further studies need to determine ac-curate viral cut off value for treatment patients at risk for PTLD.

β-globin gene cluster haplotypes are useful in diagnosis of particular molecular defects in β-thalassemia, prenatal diagnosis of β-thalassemia, and elucidating population affinities. β-globin gene cluster haplotypes were studied in 150 β-thalassemia minor and 52 healthy in-dividuals from the Fars province of Iran. DNA was extracted from leukocytes of whole blood by phe-nol-chloroform. Haplotype was determined by PCR-RFLP technique. There were 26 out of 150 with homozygous haplotypes. Haplotype I was found as the most prevalent haplotype among both patients and normal individuals. Out of 26 patients bearing homozy-gous haplotypes, 12 (46.2%) had typical haplotype I and 3 (11.5%) had atypical haplotype I. The prevalence of haplotype I in normal control subjects was around 43% (45 out of 104 βA chromo-somes). The second prevalent haplotype was haplotypes V (15.4%) and III (15.4%) for homozygous patients and controls, respectively. The most frequent mutation in patients was IVS II.1 (G→A) that was not linked to a single haplotype. IVS I.110 (G→A) mutation was linked to haplotype I. Mutation in codon 30 (G→A) was associated with haplotype V. Being Haplotype I the most prevalent haplotype in β-thal and βA chromosomes, implies that β-thalassemia mutations might have arisen in the chromosomal background common in the popula-tion, rather than due to selection pressure or gene flow (migration). Patients with haplotype IX had the highest HbF levels compared to other haplotypes.