Nitroblue tetrazolium test in patients with beta-thalassemia major

To assess the neutrophil function in thalassemia major [TM] patients and compare it with the control group, and to recognize its relevant factors. This was a retrospective cohort study, which was carried out from October 2007 to February 2008 in the Thalassemia Research Center in Boo Ali Sina Hospital in Sari, Mazandaran, north of Iran. The study population consisted of TM patients in Boo Ali Sina Teaching Hospital. The method of sampling in the case group was systematic, and it was target based in the control group. The sample size determined was based on previous studies. Thalassemia major was diagnosed based on hemoglobin electrophoresis [case group]. The control group was their brothers and sisters, who had +/- 5 years of age difference, and were of the same gender as the patients. Data collection was based on interview, investigating demographic characteristics, and also obtaining medical information from the medical records of the patients. The neutrophil function was assessed by performing nitroblue tetrazolium [NBT] reduction test. The test was carried out on both groups, and the data were analyzed by software using SPSS version13.0. In this study, 39 patients and 39 healthy controls were compared. The average age of the patients was 21.6 +/- 5.3 years, and it was 22.4 +/- 5.1 years in healthy controls [p=0.7]. There was a significant correlation between the tests' results, and the patients' age [p=0.008]. The rate of impaired NBT results in the patients was 36%, while it was 10% in controls, which were significantly different. The neutrophil activity based on NBT test was 89.9 +/- 11.6% in the case group, and 93.7 +/- 2.51% in the control group, [p=0.025]. This study indicates that neutrophil activity in thalassemic patients was significantly lower, compared to the normal control group, especially in young patients. Based on the results, evaluation of neutrophil function, and pyogenic infections in TM patients seems necessary

Determination of Hepatitis B Surface Antibody Titer in Vaccinated Children with Major Thalassemia in Kerman-Iran

Thalassemia patients, comparing to normal population are more susceptible to hepatitis, because of frequent blood transfusions. To determine the immune response of children with major beta-thalassemia, by measuring anti-hepatitis B surface antibody [anti-HBs Ab] following the last HBV vaccine injection. This study carried out on 215 thalassemic children who received three st and ard intramuscular recombinant HBV vaccines. Children age range was between 1- 4.5 with a mean age of 3.37 years. Based on the time lapsed since last vaccine injection, the subjects were divided into three groups; 0-15 months, 15-30 months and 30-45 months, respectively. Based on the serum levels of anti-HBs antibody, subjects were categorized as: good responders [anti-HBs >100 IU/Lit], low responders [anti-HBs 10-100 IU/Lit] and non responders [anti-HBs <10 IU/Lit]. The mean range of anti-HBs level in the above mentioned groups were 205.34, 128.8 and 54.25 IU/lit, respectively [P<0.0001]. In girls, the mean antibody level was 104.2 and in boys it was 95.8 IU/Lit [P>0.05]. Out of 215 selected individuals 75 [35%] were good responders, 65[30%] low responders and 75 [35%] non-responders. St and ard HBV vaccination in thalassemic children results in an immune response in more than 65% of the subjects. Therefore, assessment of anti-HBs antibody level, 45 months after the last vaccination is recommended

Detection of serologic prevalence of anti-CMV antibodies in thalassemia major patients and blood donors

Cytomegalovirus [CMV] infection has been recognized as a complication of blood transfusion. Transfusion-related CMV infection produces dramatic problems in immunocompromised patients including organ transplantation recipients, AIDS patients under immunosuppressive therapy, thalassemia major patients, and premature neonates. Regarding the importance of this infection in multitransfused patients and differences in prevalence of transfusion - related CMV infection in various reports, especially in thalassemia major patients, we decided to detect and compare the prevalence of CMV antibodies in thalassemia patients and blood donors. In this study we detected anti-CMV antibodies [IgGJgM] by Elisa technique. We tested these antibodies in 55 thalassemia major patients [45 non-splenectomized and 10 splenectomized] and 1040 healthy donors. Our results showed that anti-CMV IgG antibody was positive in 89.6% of control group and 100% of thalassamic group, thus indicating of no significant difference in these two groups. Anti-CMV IgM antibody was positive in 0.04% of control group and in 9.1% of thalassemic group showing a significant difference. The prevalence of this antibody was respectively 30% and 4.5% in splenectomized and non-splenectomized groups of patients Absence of significant difference of anti-CMV IgM antibody in patient and control group reflected high frequency of this infection in our population and also the importance of the use of leukocyte free products for high risk blood recipients. Moreover, the significant difference in anti-CMV IgM antibody in splenectomized and non-splenectomized patient groups is related to the role of spleen in clearance of infectious agents and production of IgM antibody in this organ

Human herpes virus 6 antibodies in beta-thalassemia/hemoglobin E pediatric patients.

Human herpesvirus 6 (HHV-6) is a viral pathogen that causes exanthem subitum in children. It has also been identified as the cause of life-threatening illness in immunocompromised pediatric patients and transplant recipients. We undertook a serological study of HHV-6 IgM and IgG antibody among 29 children (12 females and 17 males) with beta-thalassemia/HbE disease. The rate of infection was 86.2%; the rates of early recent infection (IgM positive only), recent infection (both IgM and IgG positive) and past infection (IgG positive only) were 13.8%, 41.4% and 31.0%, respectively. The geometric means of the IgM and IgG titers of the splenectomy group (9 cases) were 10.15 units and 11.18 units, respectively. The geometric means of the IgM and IgG titers of the non-splenectomy group (20 cases) were 10.10 units and 12.84 units, respectively. According to this study, the prevalence of HHV6 infection among pediatric patients with beta-thalassemia/HbE is very high; morever, the significantly higher titer among these patients may imply a high risk for further possible bone marrow transplantation. Increased awareness of HHV-6 infection among this population is necessary.

Immune functions in splenectomized thalassaemic children.

A prospective study to assess the immune functions in splenectomized thalassaemic children. Children were those registered in the Thalassemia major. There were 10 splenectomized children (Group 1), 10 non-splenectomized children and 6 age-matched control (Group 3). All children were shown to be HIV seronegative. The mean concentrations of serum IgG and IgA were higher in Group 1 as compared to Groups 2 and 3 but the differences were not statistically significant. Nitroblue tetrazolium (NBT) dye reduction by stimulated polymorphonuclear leukocytes was normal in both study and control groups and the differences were not statistically significant. However, NBT reduction in the unstimulated state was much higher in Group 2 as compared to Groups 1 and 3. Phytohaemagglutinin induced mitogen proliferation was normal in all 3 groups. Children in Group 1 not only had a significantly higher absolute lymphocyte count but also had a lower CD4/CD8 ratio as compared to Groups 2 and 3. Splenectomy does appear to alter the immune status of thalassemic children but the exact mechanism by which this occurrence is not clear.

Preliminary study of HLA-ABCDR antigens in CML, ANLL, thalassemia and severe aplastic anemia in Thais.

The objective of this study was to analyse human leukocyte antigen (HLA) and disease association in common blood diseases [chronic myelogenous leukemia (CML), acute nonlymphocytic leukemia (ANLL), thalassemia and severe aplastic anemia] in Thais. The subjects were patients from the Hematological Clinic, Departments of Medicine and Pediatrics, Ramathibodi Hospital who were referred for HLA typing for bone marrow transplantation (BMT) at the Histocompatibility Laboratory from March 1988 to September 1997. A total of 129 patients had complete HLA-ABC typing. The patients included 45 CML, 40 ANLL, 26 thalassemia (Thal) and 18 severe aplastic anemia (SAA). Of these, 88 patients were typed for HLA class II. The HLA class I (ABC) and II (DR, DQ) typings were performed by microlymphocytotoxicity test. It was found that HLA class I was associated with CML, ANLL and Thal, whereas, HLA class II was associated with SAA. HLA-B8 and HLA-B18 were increased in CML with R.R. values of 12.2 and 3.9, respectively, whereas, HLA-B18 was increased in ANLL with R.R. value of 4.5. In addition, HLA-DR2 and DR3 were increased in SAA with R.R. values of 3.8 and 4.8, respectively. For Thal, HLA-A2 and B46 were increased in Thal in Central Thais with R.R. values of 3.3 and 6.1, respectively, whereas, HLA-B13 was increased in Thal in Northern Thais with R.R. value of 8.5. On the other hand, HLA-B7 was absent in CML. HLA-Cw7 was decreased in CML and SAA, whereas, HLA-DR6 was decreased in ANLL and SAA. Furthermore, HLA-Cw6 was also decreased in CML, whereas, HLA-A33 and Bw4 were decreased in SAA. Although the sample size of each disease was small, the increase of HLA-DR2 was observed in SAA in Thais which was similar to other studies in different ethnic groups. These preliminary data may be useful for further study in HLA and blood disease association.

Role of splenic autotransplantation in management of thalassemia major in children

The aim of this study was to assess the outcome of pediatric patients with beta thalassemia undergoing splenectomy with splenic autotransplantation. It was conducted on 20 children suffering from beta thalassemia major over a period of two years. The rate of blood transfusion decreased considerably in both groups. One patient developed severe pneumonia in the NST group. The postoperative laparoscopic biopsy confirmed the reticular pattern of the implants with perivascular lymphocytic aggregations. All the transplanted splenic tissues showed a good uptake on scintigraphy with a gradual increase in C3, IgM and IgG levels at one and three months postoperatively with 1.5-2 fold increase of IgM and IgG levels after vaccination. Splenectomized only patients showed no significant increase in these levels

Glomerulonephritis in beta-thalassemia Hb-E disease: clinical manifestations, histopathologic studies and outcome.

We presented 8 patients with beta-thal/Hb E with glomerular diseases. Diverse glomerular lesions were seen, but diffuse endocapillary glomerulonephritis was the most common. The clinical manifestations of acute glomerulonephritis in beta-thal/Hb E differed from typical cases in the older age group, female preponderance, longer duration of edema, less hypertension, marked proteinuria, hypoalbuminemia and hypertriglyceridemia and also a longer period of recovery but their outcome was still favorable despite many risk factors of renal injury. Renal biopsy was necessary in doubtful cases to detect the correct diagnosis and give proper management. The association and mechanism of glomerulonephritis in these patients require further prospective study.

Altered T and B lymphocytes in multitransfused patients of thalassemia major.

T and B lymphocytes were estimated in 25 patients with Thalassemia major to evaluate their immune status. The percentage of T cells was significantly reduced (p < 0.001) while that of B cells was significantly raised (p < 0.001) in patients of thalassemia major as compared to age and sex matched controls.

Immunosurveillance of transfusion dependent thalassaemia and hepatitis B vaccination.

The study evaluates the probable alteration of the immune system in multitransfused thalassaemics and the modification of their immune response following administration of hepatitis B virus (HBV) vaccine. B-thalassaemics (n = 50) and EB-thalassaemics (n = 30) who received multiple blood transfusion for the chronic anaemic status had significant iron overload. They had high prevalence for hepatitis B carrier state and almost all were exposed to hepatitis B virus during the course of transfusion as shown by the positivity of hepatitis B virus markers. Thalassaemics in presence of iron overload have altered immune status in terms of depressed T-lymphocytes and raised immunoglobulins G and M. However, they showed 100 per cent seroconversion with production of antibody to hepatitis B surface antigen following Hepatitis B vaccination.

Pattern of splenic phagocytic function in Brazilian patients with sickle cell disease

The splenic function measured by the counts of pitted erythrocytes has been assessed in 87 patients with sickle cell disease (59 homozygotes for hemoglobin S (SS), 14 double heterozygotes for Hb S and beta zero thalassemia (S/beta zero thal), 4 S/beta+ thal and 10 SC patients) in Southeast Brazil. Results showed a progressive increase in pit counts according to age. The reduction pattern in the splenic function was similar for the SS, S/beta zero thal and S/beta+ thal patients, and over the age of 12 almost all patients presented counts compatible with severe splenic hypofunction. Patients with SC hemoglobinopathy presented slower development of hyposplenism and lower levels of pit counts even in advanced ages. Except for S/beta+ thal patients, the developmental pattern of hyposplenism was not different from that reported among patients in the United States and Jamaica