: Diagnosis of factor XIII (FXIII) deficiency (FXIIID) as a rare bleeding disorder is a challenge worldwide. Thus, in the present study, we used different methods including two molecular methods for detection of FXIIID. This study was conducted on individuals suspected to FXIIID. All individuals were checked by two routinely used methods of clot solubility test in Iran and two other clot solubility tests as well as FXIII activity and antigen assays. Molecular analysis was performed by PCR-restriction fragment length polymorphism (PCR-RFLP) and tetra-primer amplification refractory mutation system (T-ARMS)-PCR for only FXIIID mutation in southeast Iran (p.Trp187Arg), previously associated with severe FXIIID. Out of 151 individuals, 26 had abnormal clot solubility test with all four methods. PCR-RFLP revealed that 27 patients were homozygotes for p.Trp187Arg, whereas 12 were heterozygotes. Molecular analysis revealed that in routinely used clot solubility combinations, two homozygotes (∼8%) were missed, whereas in two other combinations, one patient (∼4%) was missed. One false positive result was observed in routinely used methods, whereas further combinations don't have false positive. T-ARMS-PCR had three discrepancies with PCR-RFLP and sequencing confirmed that the results of T-ARMS-PCR were false. FXIII antigen assay diagnosed all homozygotes, whereas in FXIII activity assay, two homozygotes had higher than 5% FXIII activity that inconsistent with severe deficiency. It seems that clot solubility test is not enough sensitive and specific and molecular analysis is the most reliable method for detection of FXIIID in areas such Iran with one or few specific mutations.

Background: Predicting future blood requirements and optimizing current transfusion practices require information on blood product utilization. Objectives: The aims of this study was to determine the utilization patterns of blood components in Zahedan, the capital city of Sistan and Balouchestan province in southeastern Iran. Methods: This descriptive study was conducted from March to August 2015. Blood bank data were collected from seven hospitals. Moreover, the archived blood request forms were studied for the demographic data of blood receivers. Results: Overall, 13,312 blood request forms (30,418 requested blood units) were studied. The mean age of the recipients was 24.3 ± 20 year and 38.9% of them were of the O blood group, followed by B (28.8%). Packed cell (PC) units were the most commonly used products (36.7%), followed by leuko-reduced red cells (25.4%), fresh frozen plasma (FFP) (16.8%), platelets (15.2%), cryoprecipitate (3.2%), and washed red cells (2.5%). The major users of blood components were the thalassemia wards (38.7%). Among the blood components, the highest wastage was related to PC with 32.3%, followed by FFP with 22.5%. The highest rate of wastage was recorded in the surgery (1,081 units) and emergency (784 units) wards for PC products and CCUs (376 units) and ICUs (167 units) for FFP products. Conclusions: A high rate of blood wastage was observed in the use of PC and FFP units. Increased awareness of physicians and medical students leads to proper blood consumption in the future. The Hospital Blood Transfusion Committee should review blood transfusion guidelines to optimize blood consumption.

Background and Aims: Rhnull, with an estimated incidence of one per 6,000,000 individuals, is an extremely rare disorder with an autosomal recessive pattern of inheritance that is more common in societies with a high rate of consanguinity. Materials and Methods: In this study, we report the first case with Rhnull, a blood group phenotype in southeast Iran, which was diagnosed during pretransfusion testing. Results and Conclusions: A 21-year-old woman with a positive parents' consanguineous marriage was found to have an unusual reaction with all packed red blood cell units during routine pretransfusion cross-match testing in the hospital. The patient's serum was reacted with all screening and identification panel cells, suspected to have an alloantibody against a common antigen or multiple alloantibodies against her absence antigens. Further studies revealed negative results for C, c, E, and e, which are highly suspected of Rhnull phenotype. Confirmatory assessments were performed, including adsorption and elution studies and Rh phenotyping of patients, along with known positive and negative controls. Due to the blood requirement of the patient, we performed serological studies on the patient's family members and found that her sister also has a Rhnull phenotype. Blood transfusion from her sister's donated units was performed, and the pregnancy was ended without any complications. Finally, due to the rarity of the Rhnull phenotype, early identification of individuals and autologous or compatible allogeneic blood transfusion should be planned prior to selective or emergency surgeries.