Iranian Journal of Blood and Cancer
Volume:6 Issue: 2, Winter 2014

Immune thrombocytopenia is a hematologic disorder characterized by low platelet count and variable bleeding manifestations due to immune-mediated platelet destruction and/or suppression of platelet production. This study was performed to evaluate characteristics and clinical presentations of adult patients with immune thrombocytopenia and to explore the clinical value of platelet antibodies assay among Iranian patients. Patients and In this cross sectional case series study 46 adult patients with immune thrombocytopenia and platelet count of < 100 x 109/L, referred to the Taleghani Medical Center, Tehran, Iran, between 2007 and 2009 were evaluated. The platelet autoantibodies were measured by means of indirect platelet suspension immunofluorescence test. According to our results, 7 patients (15.2%) displayed positive platelet antibody assay. There was a significant negative correlation between platelet count and antibody level (r = - 0.59; p <0.001). Additionally, a positive correlation between platelet count and patients’ age (r = 0.302; p =0.042) was detected. Twenty patients (56.5%) were symptomatic at presentation and the most common bleeding signs were petechia, purpura and epistaxis. Results indicated no significant correlation between increased platelet antibody level and bleeding manifestations except for hematuria (r = 0.435; p =0.02) and epistaxis (r = 0.382; p =0.015). Patients with positive antibody assay have been reported to have more bleeding symptoms and lower platelet counts. In our series, the increased antibody level was correlated with thrombocytopenia. However, among our patients with positive antibody assay no significant difference in bleeding manifestations was observed except for hematuria and epistaxis. Keywords: Immune thrombocytopenia; platelet, autoantibodies, platelet immunofluorescence test.

Systemic lupus erythematosus is a systemic autoimmune disorder with unclear etiology. The importance of some genes in the development of systemic lupus erythematosus has been implicated. The gene polymorphism in codon 72 has attracted a lot of attention and its role in the occurrence or progression of many cancers and autoimmune diseases especially systemic lupus erythematosus has been studied. In the present study we evaluated the polymorphism of codon 72 in p53 gene among patients with systemic lupus erythematosus. Patients and Expression of p53 gene was determined in lysed lymphocytes from patients with systemic lupus erythematosus who were admitted to Namazi Hospital, Shiraz, Iran, as well as 30 healthy individuals as the control group. The patients’ information, including the epidemiological profile, disease history, disease symptoms and also the laboratory findings were extracted from the hospital records. Among 77 patients with systemic lupus erythematosus, 9 (11.8%) were male and 68 (88.2%) were female. There was a significant relationship between the different allele types of p53 and systemic lupus erythematosus (p=0.033). The frequencies of Arg/Arg, Pro/Pro and Arg/Pro among normal controls were 38.8%, 28.8% and 37.5%, respectively, but among the patients, Arg/Arg, Pro/Pro and Arg/Pro genotypes frequencies were found to be 29.2%, 12.3% and 58.5%, respectively. Thus, heterozygous form of this polymorphism was shown to be associated with the disease more than the homozygous forms. There was no association between the different allele types and any of the initial manifestations of the disease and the laboratory findings. The functional oncoprotein p53 with codon 72 polymorphism may play an important role in the pathogenesis and activity of systemic lupus erythematosus. Key words: p53, systemic lupus erythematosus, polymorphism, disease activity.

Blood is permanently exposed to oxidation stress and therefore has a high antioxidants capacity. Many different factors increasing the demand for the antioxidant capacity can be observed in the stored blood of donors. Consequently, damage to erythrocytes by free radicals may occur. So it is useful to control the alternation of anti-oxidant enzymes in stored blood at different days of storage. The aim of the present study was to determine the alternation of erythrocyte superoxide dismutase and glutathione peroxidase enzyme activities in stored blood. Blood samples were obtained from 67 donors with average age of 26 years. Samples were collected in CPDA-1 anticoagulation solution. Erythrocyte superoxide dismutase and glutathione peroxidase enzyme activities were determined using Kei Satoh method and kits from Randox. The measurements were performed at the days 1, 7, 14, 21, 28 and 35 of storage. The blood bags were stored after each sampling at 4◦C. Data were analyzed using analytical variance statistical test and SPSS version16 software. The erythrocyte levels of erythrocyte superoxide dismutase and glutathione peroxidase enzyme activities decreased significantly at the day 14th (P<0.001) of storage compared to day 1. Our results suggest that during blood storage, antioxidant defense in erythrocytes were depleting gradually depending on the day of storage. Based on our finding a 14 days period can be considered a safe storage limit for transfusion in relation to oxidative stress on the RBCs in storage medium. Key words: Red blood cell, oxidative damage, antioxidant enzymes, blood storage.

In this study we have prepared the IgA solution from normal human plasma using plasma fractionation and ion exchange chromatography. Using fractionation of plasma with cold ethanol (starting with 8% ethanol), fraction III was prepared as a suitable source for IgA preparation. Then it was treated with caprylic acid for separation of impurities. For enrichment of IgA, ion exchange chromatography using Sephadex DEAE A-50 on a Pharmacia glass column was carried out. By fractionation of pooled human plasma and then treatment with caprylic acid, the amount of immunoglobulins showed an increase from 17% to 80% measured by immunoturbidimetric method. Then the ion exchange column chromatography by Sephadex DEAE A-50 was carried out. In washing solution 65 % of loaded IgG and only 17% of loaded IgA were found. In elution solution 32% of loaded IgG and 79% of loaded IgA were obtained, which showed the ion exchange chromatography is a promising method for preparation of enriched IgA solution. Because of the similarity between IgA and IgG, techniques based on net charge and molecular differences would not give a high yield in IgA isolation. As IgA has a negative charge at the pH 8.1, Sephadex DEAE A-50 which is able to bind proteins at relatively high ionic strength was chosen for ion exchange chromatography. After column ion exchange chromatography, the concentration of obtained IgA was three times more enriched in comparison with the concentration of IgA in normal plasma.

Children with idiopathic thrombocytopenic purpura who are treated with intravenous immunoglobulin therapy might experience a decline in their absolute neutrophil count. The aim of this study was to investigate the incidence of neutropenia following intravenous immunoglobulin therapy in children with idiopathic thrombocytopenic purpura undergoing intravenous immunoglobulin therapy. Patients and This was a retrospective cross sectional study. Patients with idiopathic thrombocytopenic purpura admitted to Ali-Asghar hospital from October 2003 to June 2010 with no immunosuppressive diseases negative coombs test and no sign of other infections before the admission entered the study and their neutrophil count before the intravenous immunoglobulin therapy, and in days1, 2, and 3 after the initiation of therapy was recorded. From eighty nine patients 51 patients (57.3%) were male and 38 patients (42.7%) were female. Neutropenia was seen in 13 patients (14.6%) after treatment, but there was no statically significant difference between the mean absolute neutrophil count before and 1, 2, and 3 days after the start of the treatment (P=0.922). Intravenous immunoglobulin can lead to neutropenia in a few number of patients which is transient and self limited and most patients get benefits from intravenous immunoglobulin therapy as induced platelets count.

Blood transfusion centers are under considerable pressure to produce platelet concentrates with a shelf life limit of 3 to 5 days. Many approaches have been investigated experimentally to produce new hemostatically active platelet products that are capable of long term storage. In this article infusible platelet membrane will be explained as a platelet substitute versus conventional liquid-stored platelet concentrates with regard to their benefits and disadvantages in transfusion medicine. This review shows that lyophilized infusible platelet membrane as a platelet substitute might offer many important benefits over common platelet concentrates with few disadvantages. Infusible platelet membrane may have efficacy, safety and acceptable tolerability without thrombogenecity, immunogenicity or toxicity. The other main benefits of this product are improved shelf life, ease of storage, high-precision dose calculation, easy reformulation, reduced viral and bacterial load, decreased refractoriness to platelet transfusion, reduced contaminating red and white blood cells, reduced side effects due to removal of undesirable effects of intracellular and extracellular proinflamatory mediators and removal of platelet-derived microparticlecs as a source of CD40/CD40L ligands, which can enhance post-transfusion reactions, achieving hemostatic response without increasing the circulating platelet count, not being removed from circulation by immune mechanisms or sepsis and not requiring blood typing. In spite of these benefits, there are still some difficulties in demonstrating its efficacy, short-term circulation and hemostatic function. Therefore, further human clinical studies will be needed to fully define the exact role of infusible platelet membrane in the management of patients with thrombocytopenia.

Thrombotic thrombocytopenic purpura (TTP) is the most malignant variant of microangiopathy that usually presents by typical symptoms including thrombocytopenia, hemolytic anemia, neurological abnormalities, fever and renal impairment. Report of the Case: We report a 12-year-old male presented by cytopenia, fever, purpura on his extremities, seizure and lethargy. Peripheral blood smear revealed low platelet and increased schistocyte, but renal function tests were normal. Final diagnosis of TTP was confirmed by measuring ADAMTS-13 autoantibody (> 60). Atypical TTP can presents without renal impairment. In these cases the measurement of ADAMTS-13 activity as a specific test as well as ruling out secondary TTP should be considered.