Iranian Journal of Blood and Cancer
Volume:1 Issue: 1, Autumn 2008

Thalassemia syndromes are the most common genetic disorders in the world. They happen due to genetic defects in process of haemoglobin synthesis, and would be classified to many groups mainly α and β, based on the kind of defect. Anemia is the main clinical manifestation of this phenotype of disorder. In order to correct the chronic anemia in thalassaemic individuals, they may need to get 4-6 blood units per month. They are also prone to blood borne infections. Patients and We retrospectively evaluated medical records of 360 major β- thlassemic patients in northeastern Iran to find the viral blood borne status among them. The findings showed that 209 (58.8%) were male and 151 (41.94%) were female. About blood borne viruses, 30 (8.33%) had positive result for anti-HCV. Eight persons (2.22%) had positive HBsAg. Twenty-two persons (6.11%) had positive HTLV-I serums. No one had positive HIV (1/2). Seven (1.94%) were positive for both HTLV-I and anti-HCV. Two ones (0.55%) had positive serums for both HTLV-I and HBsAg. Similar to other geographic regions, the HCV was the most common type of viral infections in northeastern Iran. Surveying the HTLV-I, the northeastern part of country is one of endemic sources for the HTLV-I and in fact 22 individuals showed it. Also HBV had infected part of major β- thalassemic patients. Fortunately there was no case with the HIV that intensified the blood safety of blood transfusion organization.

Thalassemia is common in Iran. Appropriate therapy for this disease includes a regular blood transfusion and chelation therapy. However, patients will inevitably confront with side effects, particularly iron overloads in critical organ including heart, ductless glands and liver. This study tries to determine the prevalence of diabetes mellitus in transfusion dependent β thalassemia major. Patients & This is a cross sectional study and included all 437 patients suffering Thalassemia major that were referred to medical centers linked to the Iranian blood transfusion organization since Jan 2004 up to Jan 2005. All patients tested for CBC, FBS, 2hr BS, HbAlC, liver and renal function, and endocrine disease. Initially, reports of adenoidal experiments as well as other associate parameters were provided via medical folders. Four hundreds and thirty-seven patients enrolled in this study, the diabetes found in 28 patients (5.4%). There was no relationship between sex and the kind of Thalassemia. In patients with Thalassemia intermedia, the mean of age was 34.7 ± 1.4 years and the mean of ferritin was 893.2 ± 122.4 Mg/dl. and patients with Thalassemia major, were 27.1 ± 0.3 years and 1678.8 ± 87.5. Mg/dl respectively the mean of age and ferritin in two groups of Thalassemia intermedia and Thalassemia major had significant difference while the mean of ferritin in diabetic and non-diabetic groups had no significant difference. We can conclude According to previous studies considering endocrine abnormalities in thalassemic patients, that our data is just like others.

Pediatric oncology patients frequently need several painful procedures during their diagnostic and therapeutic process. During the past decades, most centers abroad have developed safe and effective protocols for procedural sedation and analgesia in children. This report describes procedural sedation and analgesia as performed in pediatric oncology unit and tries to report success of sedation and incidence of complication. Between 1998 and 2004, all invasive procedures for oncologic children presented in Aliasghar and Dastgheyb hospitals in Shiraz, were performed with deep sedation or general anesthesia and the failure(s) and side effects were evaluated. totally 1381 deep sedations or general anesthesia were carried out and 1792 invasive procedures were performed For 166 children (103 boys and 63 girls) with suspected or proved oncologic disease with age range of 1.5 to 15 years Lumbar puncture (LP) and intratechal injection were performed in 73% of patients mean while bone marrow aspiration and biopsy were performed in 26% and 1% of patients; respectively. The main side effects were nausea and vomiting in about 7% of patients. No failure of the invasive procedures especially LP happened. Use of general anesthesia and/or deep sedation on pediatric patients with oncologic disease is safe and can be recommended in these patients.

Leukocytes causing a wide variety of side effects after transfusion are present in all blood products prepared by standard methods. As a consequence, the use of filter technology for leukoreduction has been widely practiced. According to AABB accreditation in 1996, leukoreduced blood components must contain less than 5×106 leukocytes per unit, but sometimes this value is higher even in leukoreduced products. In this study we did absolute leukocyte count in filtered (home made bedside filter) packed cell units by two methods of true count as standard method and CD45 MoAb. 93 packed cell units were stored at 4°c and filtered by two types of home-made filters according to manufacturer''s instructions. Furthermore, eight packed cell units were filtered by Europe certified control group filters (bio-fil). Sample preparation was done according to True count kit and CD45 MoAb procedures and analysis was performed by flowcytometry (EPICS-XL,coulter) and (Partec PAS III).The results were then analyzed by chi2 test via SPSS. The mean values of leukocyte count/unit by anti CD45 and True count method were 9×106 and 10×106 respectively in 55 pre-revised filter bags; these figures were 4.2×106 and 4.8×106 in 30 post-revised filters, whereas the mean leukocyte count/bag in eight control filters was 2.3×106,we selected randomly eight test units out of 53 and 30 pre-revised and post-revised filtered bags, respectively(equal with number of units in the control group) to compare the test and control groups The mean values of leukocyte count/bag in pre-revised test group was 7.9±5.4×106 and in postrevised test group was 4.2 ×106 but in the control group it was 2.3 ×106 (p value<0.05). According to the results, the mean leukocyte count/bag in pre-revised group was higher than AABB standard. 38.2 % of bags had lower and 61.8 % had higher leukocyte count than the standard value (48.9 to 74.6 % with CI=95 %), this indicates the necessity of revision in product technology of homemade filters. relevant manufacturer revised the product technology and material accordingly, so that new filters (post-revised group) reduced leukocytes within standard limits (leukocyte count in 6 bags were out of standard range).In post-revise filter group, 20 % had a leukocyte count of more than 5×106 while 80% showed less than this value (5.7%-34.3% with CI=95%).There is a significant difference between control and pre-revised test groups (p=0.03).In post-revised test group, despite significant differences, the mean values of leukocyte count/bag were within normal standard range. The results of this research caused home-made filter production with higher quality.

the Iron deficiency anemia (IDA) is a common cause of referral to medical departments. Chronic gastrointestinal (GI) blood loss is important cause of IDA and also an alarming sign of serious underlying disease. The information concerning ideal evaluation of the gastrointestinal tract in patients with the IDA is scant. We designed a prospective study to determine the frequency of GI tract lesions in patients with the IDA. This cross - sectional study was conducted since January 2006 to January 2008 at Imam Hospital, Urmia in the west of Iran. The study population was comprised of 227 patients including men over 40 years and post menopausal women with probable IDA. After identification of patients, we extracted detailed data via medical records, history taking, physical examination and laboratory. Data was analyzed by the chi square and student’s t-test. the patients totally underwent 478 in: 214 upper GI endoscopies, 179 colonoscopies, and 85 bariumenema. The mean age of subjects was 61 years (SD±15), and 55% were women. Upper GI lesions (esophagitis, gastritis, peptic ulcer and gastric cancer) accounted 32.1 (73 case) percent of cases. Colonoscopy findings include: polyps of colon were found in 10 (4.4%) of cases and colon cancer in 15 (6.6%). Etiology of IDA was not detected in 126 (55.5%) of our patients. We concluded despite relatively low rate of malignant gastrointestinal lesions that were found in our study, GI tract assessment is necessary in iron deficiency anemia, chiefly in the patients with symptoms related to upper or lower gastrointestinal tract.

Thalassemic patients are at risk of zinc deficiency due to various causes including desferal injection, hyperzincuria, high ferritin levels, and hepatic iron overload. We evaluate the effect of zinc supplementation on linear growth of beta-thalassemia patients. one-hundred beta-thalassemic major patients whose heights were within 3rd to l0th percentile were randomly divided into two groups, each group consist of 50 patients: Group I received oral zinc (60 mg per day) and Group II served as control group without zinc supplement. Patients were followed for 18 months and we control height. Data was analyzed by SPSS 11.5 software by nonparametric and T test. The mean age and height of the patients in Group I were 8.14±1.30 year and 120.83 ±6.41cm, and in Group II, 8.27±1.14 year and 121.85 ±6.18cm, respectively. Eighteen months later the mean height was 125.14±6.17 cm in Group I and 126.1±6.07 cm in Group II (P = 0.464).No statistically significant difference in height was noted between the two groups after a period of eighteen months (P=0.464). The results of the research revealed that whereas the role of zinc has been proved in the growth process. It is concluded that oral zinc sulfate has no significant effect on linear growth of beta-thalassemia patients.

It is suggested that HA-1 mismatching among hematopoietic stem cell transplanted recipients-donors is associated with acute graft-versus-host disease (aGVHD). So the aim of this study was to evaluate HA-1 frequency and examine the correlation between HA-1 disparity and GVHD patients who received transplantation from a HLA-identical sibling.Samples and the DNA was extracted from 30 pairs of HLA-A2-positive Iranian recipients-donors with GVHD I-IV and 25 pairs without GVHD. All the patients received HSCT from HLA-identical siblings. HA-1 was detected by SSP-PCR method. The HA-1 typing was performed using SSP method (SSP Minor Histocompatibility Antigen primer sets). The frequency of HA-1R and HA-1H alleles in patients were 0.55 and 0.45 respectively, and showed no significant difference with these alleles frequency in donors 0.53 and 0.47 respectively (p>0.05). The HA-1 disparity was detected in 8 out of 55 donor/recipient pairs (14.5%). The aGVHD (grades I-IV) occurred in 6 (75%) out of 8 patients but in spite of higher incidence of it in the group of patients with HA-1 incompatibility, it was not statistically significant. In spite of higher frequency of HA-1 disparity in GVHD+ group our data did not reflect a significant association between the HA-1 disparity and risk of acute GVHD.

The Hypertension is divided into two types: primary and secondary. the secondary type, is particularly due to renal and arterial origin and is mostly seen. In children the Secondary hypertension caused by malignancies is rare. This is a case of abdominal rhabdomyosarcoma with malignant hypertension.