Iranian Journal of Blood and Cancer
Volume:12 Issue: 4, Dec 2020

Thrombotic Thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathic disease, associated with thrombocytopenia and hemolytic anemia. It is caused by an enzymatic dysfunction responsible in cleavage of blood clotting factors. In this study we have tried to review the available approaches in diagnoses of the disease as well as treatment strategies. Based on the what the current review has provided, treatment policy depends on accurate diagnosis, it seems that developing a diagnostic and treatment guide for this disease is currently essential. Also, it’s necessary to study genetic types of TTP with more details because, hopefully, new techniques of gene therapy open a window to more stable treatments.

RhD antigen system is the leading cause of hemolytic disease of the fetus and newborn (HDFN). Paternal molecular RhD zygosity test is valuable to decide on the use of anti-D immunoglobulin prophylaxis in Rh D-negative pregnant women. We aimed to investigate the paternal RhD zygosity by two molecular methods among blood donors in Kurdistan province, the west of Iran. We also compared these two methods in determining RhD zygosity.

100 RhD positive blood samples were collected from male blood donors with RhD negative spouses who were referred to Kurdistan Blood Transfusion Center. The phenotype of all samples was tested for Rh D, C, c, E and e antigens by standard hemagglutination methods. Then, RhD zygosity of all samples was evaluated in terms of Rhesus box marker by SSP-PCR and PCR-RFLP methods. 

Among 100 RhD positive samples, 37% were heterozygote and 63% were homozygote for RhD gene. Both SSP-PCR and PCR-RFLP methods were able to detect zygosity with similar accuracy. Moreover, Rh phenotyping revealed that DCCee (38%) and Dccee (2%) were the most and the least frequent phenotypes in our sample, respectively. 

RhD zygosity determination in men who have an RhD negative partner by molecular methods such as PCR-SSP and PCR-RFLP could be the first step in preventing HDFN and avoiding unnecessary administration of Rh immunoglobulin in Iran.

Bleeding assessment tools are key components in the evaluation of patients suspicious for bleeding disorders. The exact determination of the normal ranges of ISTH-BAT (International Society on Thrombosis and Hemostasis –Bleeding Assessment Tool) in the healthy population is a crucial step for determining who needs to be referred for further coagulation laboratory examinations. We aimed to determine the normal range of ISTH-BAT and simultaneously evaluate the Von Willebrand disease (VWD) diagnostic panel tests in a healthy Iranian group.

ISTH-BAT as well as prothrombin time, activated partial thromboplastin time, factor VIII clotting assay, von Willebrand factor (VWF) antigen, VWF ristocetin cofactor assay, ristocetin induced platelet agglutination, and blood group typing were assessed for 25 normal adults without any bleeding symptoms or a known coagulation disorder.

In the 25 studied subjects (13 men, 12 women), the range of bleeding score was 0-6 in women and 0-5 in men; however, since the scores were lower than 2 in most (68%) cases, the interquartile range (IQR) was used for normalizing the data. The ISTH-BAT normal range was found to be 1-4 in women and 0–2 in men.

According to this result, the ISTH-BAT cut-off for abnormal bleeding score was ≥5 in adult women and ≥3 in adult men. These data may be valuable in the routine practice of clinicians and adult hematologists in our country for the assessment of individuals with suspected bleeding disorders.

The well-known toxic effects of benzene toxicity are bone marrow depression, reduction in blood cell counts, and induction of leukemia and aplastic anemia. This study was designed to evaluate biomarkers of aging in red blood cells (RBCs).

Mice were exposed to benzene (50, 100, and 200 mg/kg/day) orally for 28 days. A group of benzene-exposed mice were injected intraperitoneally with N-acetylcysteine (NAC, 150 mg/kg/day). Hematological factors, erythrocyte morphology, and sialic acid content of RBCs along with oxidative stress biomarkers were investigated. 

Benzene dose-dependently reduced RBCs count, hemoglobin level, RBCs membrane sialic acid levels, the total antioxidant capacity of plasma, and G6PD activity of RBCs. The activity of antioxidant enzymes and lactate dehydrogenase, oxidative damage end-products and bilirubin levels, reticulocyte count, and RDW and MCV ranges increased in a dose-dependent manner. Poikilocytosis (spherocyte, burr cell, schistocyte and blister cell) and anisocytosis were observed in high doses of benzene. 

Our results support the acceleration of RBCs aging and hemolytic anemia in mice exposed to benzene. Co-administration of NAC as an antioxidant effectively alleviated hematotoxicity of benzene.

Gastrointestinal Stromal Tumor (GIST) is the most common mesenchymal tumor of the gastrointestinal tract and most commonly affects the stomach, while fibromatosis is a rare locally aggressive fibrous tissue neoplasm. There have been reports of GIST and fibromatosis occurring in same individual and in most of them fibromatosis occurs within the abdomen. In 75% of patients fibromatosis occurs after the diagnosis of GIST while in 20% it is seen synchronously. Here we report a case of axillary fibromatosis with synchronous GIST of the gastroesophageal junction in a 45-year-old male treated with surgery and now on adjuvant imatinib.