Iranian Journal of Blood and Cancer
Volume:4 Issue: 2, Winter 2012

Human β-like globin genes regulaon during development from embyonic to adult stage results in generaon of different types of hemoglobin with different funcons. As β-thalassemia and sickle cell disease are disorders of β-globin chain, epigenec drugs such as thalidomide and sodium butyrate which can induce γ-globin gene are considered as a novel therapeuc approach. Drugs effecve in decreasing DNA methylaon and alteraon of histone methylaon pa$ern can result in γ-globin gene upregulaon. This study was performed on erythroid progenitors derived from cord blood CD133+ cells. Erythroid progenitors were treated with thalidomide and sodium butyrate as single and combinaon therapies in 10 μM concentraons. Chroman Immuno Percipitaon (ChIP) assay was used to evaluate the change in H3K27 methylaon pa$ern. Also, Real-me PCR assay was used to compare the number of DNA fragments resulng from immunoprecipitaon in different drug treatment groups. Real-me PCR assay indicated considerable effect of thalidomide single therapy in decreasing H3K27 methylaon compared with sodium butyrate and combinaon therapy. According to the results of this study, it seems that the synergisc effect of thalidomide and sodium butyrate combinaon therapy on γ-globin gene inducon arises from other epigenec mechanisms.

A simple inexpensive con!nuous quality control method, by means of eight basic blood coun!ng parameters, obtained from automated hematology analyzers, using pa!ent samples, is described. A few samples with low, normal and high values were selected and introduced to the instrument early in the morning as the first count, the results of which are plo#ed on the appropriate chart as dots. The same samples were again given to the instrument at noon and the results were plo#ed on the same chart as the arrow heads. An arrow is then drawn from each pair of consecu!ve counts. The same procedure is repeated every 6 hours, using a newly selected set of samples, and the last sample which is selected in late evening on each day, is introduced to the instrument on the next morning. Hence pa!ent samples are run at the beginning and the end of three daily work shi$s. An explana!on is given to use the direc!on of the arrows as the main factor to assess the quality of the instrument performance. This method can be easily applied to any hematological laboratory and can simply be performed even by laboratory technicians.

The double heterozygous state of α/β thalassemia may alter the hematological indices and modify the phonotype. In addion, definite characterizaon of co-inheritance of α- and β-thalassemia heterozygous carriers may change the process of genec counseling. An Iranian couple with low hematological indices was analyzed for α-globin gene deleons using mulplex gap PCR method and β-globin gene mutaons by ARMS-PCR method and DNA sequencing. The -20.5kb α-globin gene deleon was found in both individuals, and the IVSI-110(G>A) mutaon in β- globin gene in male partner. The β -globin gene sequence was normal in female partner. Therefore, the couple was informed about the risk of having fetuses with hemoglobin Bart’s hydrops fetalis. The co-inheritance of α/β thalassemia should be considered in genec counseling of families screened for β-thalassemia major prevenon.

Coagulaon factor XIII gene, protein structure and funcon Coagulaon factor XIII (FXIII) is a tetrameric (FXIII- A2B2) pro-transglutaminase enzyme with an essenal role in the final stage of coagulaon cascade by cross linking the fibrin monomers and stabilizing the fibrin clot. Congenital FXIII deficiency is a rare bleeding disorder, with an autosomal recessive trait inheritance, and a frequency of about 1 in 2 million people. Most cases of FXIII deficiency are associated with FXIII-A subunit deficiency and only few FXIII-B subunit deficiencies have been reported. Severe FXIII-A deficiencies are associated with some moderate to severe clinical complicaons including umbilical bleeding during infancy, impaired wound healing, pregnancy loss in affected women, life-threatening intracranial bleeding and also subcutaneous and so# ssue bleeding. Diagnosis of FXIII deficiency can be achieved by clot solubility tests in 5 M urea or 1% monochloracec acid as a screening assay, and also quantave evaluaon of the acvity or angenic levels of FXIII A and B subunits. There have been recommendaons for primary prophylaxis or replacement therapy in FXIII deficient paents, in order to prevent spontaneous bleeding, bleeding during minor and major surgeries, or prevenon of pregnancy loss in women. Acquired FXIII deficiency has also been reported as a result of decreased producon or high consumpon of FXIII as well as the secreon of autoanbodies against FXIII subunits.

Introducon: Acute hemorrhagic edema of infancy (AHEI) is a cutaneous leukocytoclas! c vasculi! s of infants, clinically characterized by acute development of peripheral edema and targetoid purpuric lesions on face and extremi! es. It is considered to be an uncommon form of cutaneous vasculi! s occurring in children younger than 2 years old. The clinical picture has a violent onset, a short benign course followed by spontaneous complete recovery. Case presenta! on: We describe two males with acute hemorrhagic edema. In one case the disease appeared a«er upper respiratory tract infec! on. AHEI is a benign disorder despite its drama! c appearance. The most striking classic features of the disease are the contrast between the acuteness of the cutaneous lesions and the good general condi! on of the pa! ent. Considering its clinical features, AHEI can be jus! fiably characterized as a unique disorder, dis! nct from Henoch–Schenlein Purpura (HSP).

Hematologic abnormali «es are generally present among systemic lupus erythematosus pa» ents. Idiopathic thrombocytopenic purpura can be the first manifesta«on of SLE, followed by other symptoms and signs of disease appearing several years later. Although bleeding due to immune thrombocytopenic purpura is usually mild and occurs in mucocutaneous surfaces, but it may be severe and represent in unusual sites such as an ovarian cyst. We report a case of SLE presented with immune thrombocytopenia and a large hemorrhagic ovarian cyst.

To explore a possible rela!onship among blood groups and the prognosis of breast cancer, this case- control study was carried out in Arak city, Arak province, Iran. One hundred and thirty four pa!ents with breast cancer were inves!gated. ABO blood groups were obtained from medical records. Mul!variate analyses were performed, including size of tumor, axillary lymph nodes involvement and prognosis of the pa!ents. We found that there is a significant rela!onship between the blood type and the size of tumor (P.V=0.035), axillary lymph nodes involvement (P.V=0.001) and the prognosis of the breast cancer (P.V=0.014). The blood type of among our pa!ents with breast cancer seems to be a prognos!c factor and the presence of B-an!gen shows associa!on with poor prognosis of breast cancer.

The human T-cell lymphotropic virus type I is the first retrovirus idenfied in humans. The virus has been associated with adult T-cell leukemia/lymphoma, human T-lymphotropic virus type I, myelopathy/tropical spasc paraparesis, uveis, arthris, pulmonary lymphocyc alveolis, keratoconjuncvis sicca, and infecous dermas. Human T-lymphotropic virus type Iis endemic in Japan, parts of central Africa, the Caribbean basin and South America, Melanesia and Iran (city of Mashhad). The aim of this study was to evaluate serological prevalence of human T-lymphotropic virus type I/IIinfecon among blood donors, major thalassemic paents and individuals infected with hepas B and C viruses in the city of Isfahan, Iran. Sera were collected from 140 blood donors (440 samples), 150 major thalassemic paents, 150 and individuals with hepas B and C and were tested for the presence of human T-lymphotropic virus type I/ IIspecific anbody in a 6 months period in 2007using ELISA and Western Blot tests Blood donors tested were negave for human T-lymphotropic virus type I/IIinfecon, but in major thalassemic paents including 88 males and 62 females, 5 were posive (3.3%). Stascal analyses did not show any significant difference between genders. In individuals with hepas one was border line (HCV). The results indicate a low level of coinfecon of human T-lymphotropic virus with HCV in this part of the country. However, the observaon of a case with borderline findings, among this group and also posive cases among thalassemic paents, suggests the presence of the virus in blood donor populaon so this virus could be present in Isfahan but more invesgaon is needed to evaluate the need for screening tests to detec human T-lymphotropic virus type I among blood donors in Isfahan.connuing screening and educaon of parents and also mass media educaon against consuming “Fava bean” and “Naphtalen”.