Red Blood Cell Immunization and Contributing Factors in 685 Thalassemia Patients.

Background: An analysis of red blood cell alloimmunization in patients with thalassemia can help to devise specific strategies to decrease the alloimmunization rate. This study explored the frequency and specificity of alloantibodies and autoantibodies against red blood cell (RBC) antigens in patients with thalassemia referring to the Iranian Blood Transfusion Organization (IBTO) Immunohematology Reference Laboratory (IRL) in Tehran. Materials and Methods: This study first examined the laboratory records of 23,113 patients suffering from different diseases referring to IBTO's IRL for pretransfusion testing in the 2008-2015 period. ABO and Rh(D) typing and antibody screening tests were performed for all 23,113 patient records and 685 (2.97%) beta-thalassemia patients with positive pre-transfusion test results (antibody screening and/or DAT) were selected for further investigation. Results: The antibody screening test was positive in 640 out of 685 thalassemic patients (93.4%). DAT was performed for 529 patients, 226 (33%) of which showed positive results. Meanwhile, 161 out of 685 beta-thalassemia patients (23.5%) had positive auto control test results, reflecting the possible presence of allo- and/or autoantibodies. The most common antigen-specific alloantibodies were directed against K and E RBC antigens with a frequency of 25% (Anti-K) and 11.91% (Anti-E), respectively. The development of two antibodies (double antibodies) in one patient was observed in 80 individuals (11.46%). Conclusion: Age, gender, history of pregnancy, and splenectomy were not contributing factors to the antibody presence in the patient population under study. Extended red blood cell phenotyping should be considered as an essential procedure for expected multi-transfused thalassemia patients before blood transfusion. Considering the high frequency of anti-K and anti-E observed in this study, it is recommended that thalassemia patients in Iran are tested through phenotyping of RBC units for K and E antigens before transfusion.

The First Comprehensive Study of H-Deficient Phenotypes in Iran.

BACKGROUND: The lack of correct blood grouping practices can lead to missing of the rare Bombay Oh phenotype and subjecting patients to the risk of severe hemolytic transfusion reaction. In the absence of blood donor registry, transfusion management of patients is a challenge. We performed this study in order to estimate the prevalence of the Bombay blood group (Oh) in Iran and to determine whether consanguinity plays a role in the prevalence of Oh group. METHODS: This is a descriptive study in the Immunohematology Reference Laboratory of the Iranian Blood Transfusion Organization (IBTO) Tehran, Iran, over a period of 7 years. All donor blood samples showing blood group O and a strong initial reaction with blood group O RBC control cells were tested with anti-H lectin. Also blood samples from blood group O patients were tested with anti-H lectin if all cells on both antibody screening tests and antibody identification panels were reactive with negative auto control test. Specialized tests like adsorption/elution technique and inhibition assay for determination of secretor status were performed on Oh cases. Any history of consanguineous marriages were recorded. All variables were categorical variables, and percentage and proportions were calculated manually. RESULTS: Analysis of the results of over 7 million first-time blood donors in Iran showed that the most common ABO blood group was O, with 2,520,000 (36%) subjects. 56 Oh individuals' (donors and patients) phenotypes (0.0008%) were detected. Consanguinity was observed in 50 cases (89%). CONCLUSIONS: This study shows that the prevalence of Bombay blood group in the general population of Iran is relatively high (0.0008%) and associated with consanguineous marriage. Thus, consanguinity is still an important risk factor present.

First report of the rare RhCE-depleted D--phenotype in sixteen people of Iranian origin.

BACKGROUND AND OBJECTIVES: In transfusion medicine, it may be a challenge to acquire compatible blood for patients who have clinically important alloantibodies to high-prevalence antigens. The aim of this study was to study prevalence of rare D-- phenotype in samples from patients and their relatives referred to the Immunohematology reference laboratory of the Iranian Blood Transfusion Organization and the detection and identification of the phenotype and associated antibodies, particularly in an antenatal setting. This is the first report of the cases evaluated by the IBTO and family studies of the D-- proposita in Iran and possibly the first attempted comprehensive study in the current transfusion-related literatures. MATERIALS AND METHODS: This retrospective cross-sectional study was carried out on 6720 pregnant women and individuals with difficult positive pretransfusion testing referred for ABO/Rh(D) typing and antibody screening during a period of 8 years from 2008 to December 2016 in the Immunohematology Reference Laboratory of the Iranian Blood Transfusion Organization, Tehran, Iran. RESULTS: During 2008 to December 2016, 16 persons from ten families were detected to have rare D-- phenotype. Anti-Rh17 and anti-c were identified in plasma of the 11 persons, including 10 females with a history of multiple unsuccessful pregnancy and the total number of 24 abortions and one male with history of blood transfusion vs. 5 individuals, including an unmarried single woman, 1 person with a history of first-time pregnancy and 3 persons with a history of multiple pregnancy, who showed no alloimmunization. Based on these collective findings, we interpreted these results as being confirmed as D-- phenotype (0.23%). CONCLUSION: Irrespective of Rh (D) group a serological antibody screening test is recommended to be required in a National prenatal testing guideline.

Comparison of the Effects of Video Modeling and Clinician Modeling on Naming Deficiency in Aphasic Patients.

BACKGROUND: Aphasia is the most frequent disorder that could occur following a stroke. Aphasia has a negative impact on the patient's communication ability through language. One of the common consequences of aphasia is naming deficits that can lead to communication disorders. Therefore, the treatment of aphasia is necessary. The aim of the current study was to investigate the effect of video modeling and clinician modeling on naming skills of patients with chronic aphasia. MATERIALS AND METHODS: The design of this prospective single subject study was ABA that performed on four patients with chronic aphasia. participated. This study was administered during three phases including the baseline (three sessions); the intervention (nine sessions); and a follow-up phase (three sessions). The outcome measure was taken in three phases including baseline, intervention, and follow-up. For each patient, the naming score for items modeled by the clinician, the naming score for items modeled video modeling by other, the naming score for self-video modeling, and the reaction time score were recorded. RESULTS: A total of three patients complete the study and one of them refused to continue treatment. The naming score of all modeling types increased in all patients. In the other words, the intervention helped the patients be improved in naming. Also, the results of the reaction time indicated that the video modeling, as well as clinician modeling, could decrease the response time that means the intervention could increase the speed of retrieval processes. CONCLUSION: In our study, all three types of modeling could improve the naming scores in patients with chronic aphasia. Additionally, the findings demonstrate that the clinician and video modeling might increase mental processing for naming verbally.

Effects of multimodal communication program on patients with chronic aphasia: a single-subject A-B-A design study.

BACKGROUND AND AIM: Aphasia as a common consequence of stroke, is an acquired neurologic communication disorder that can affect symbol language processing. Different types of intervention approaches have been introduced. Multimodal Communication Program (MCP) is a new augmentative alternative communication approach in chronic aphasia. The aim of this study was to investigate the effect of MCP on communication skills of patients with chronic aphasia. METHODS: This prospective, single subject, A-B-A design study was done during 2016 in Semnan, Iran. Participants were two patients with severe aphasia with a single left-hemisphere stroke. Three phases, including baseline, intervention and follow-up were administered. The patients received nine-hour intervention, over 10 working days. RESULTS: Three different scores were calculated for each patient: verbal efforts, the frequency of each modality and the accuracy of switching between modalities and the reaction time. The frequency of verbal modality increased for both patients. They could switch between modalities more successfully than before the intervention. The results for the reaction time, however were challenging. The onset reaction time decreased for patient 1, and increased during switching between modalities, and patient 2 showed the opposite. CONCLUSION: The MCP can improve the communication skills in patients with chronic post stroke aphasia. However, some factors, such as reduction of the patients' reaction time is probably related to the amount of allocated resources during intervention. TRIAL REGISTRATION: The trial was registered at IRCT center with ID: IRCT2016032325194N3. FUNDING: The study was financed by Semnan University of Medical Sciences (Grant no.: A-10-333-3).

First Report of Known Rare Rhnull Phenotype Individuals in Iran.

Rhnull phenotype is a rare blood group with a frequency of approximately 1 in 6 million individuals, transmitted via an autosomal recessive mode. It is characterized by the weak (Rhmod) or lack (Rhnull) of expression of all Rh antigens on the red cells. The clinical significance of its assessment is that such patients with Rhnull syndrome are associated with chronic hemolytic anemia of varying degrees. Another clinical importance is that such subjects readily form alloantibodies when exposed to Rh antigens.We report herein a rare Rhnull phenotype in a sibling, which was detected as a part of the difficult sample work-up for red cell antibody screening and identification.

Health-related quality of life in Iranian adult men with severe hemophilia.

: Health-related quality of life (HRQOL) assessment should be part of the regular clinical assessment of persons with hemophilia. This study assessed quality of life of severe hemophilia patients (Type A) in Tehran comprehensive hemophilia care center. This cross-sectional study was done in 2016 and 84 men aged over 20 years with severe hemophilia (Type A) were assessed. All patients have been treated over 10 years at the comprehensive hemophilia care center, Iran. The HRQOL assessment includes the A36 Hemophilia-QOL questionnaire, sociodemographical and clinical characteristics. The overall HRQOL mean (±SD) in the present sample was 69.1 ±â€Š26.0 (A36Hemofilia-QoL Global Index). The mean (±SD) scores according the domains were: physical health (15.0 ±â€Š5.7), daily activities (6.7 ±â€Š3.8), joint damage (4.2 ±â€Š2.9), pain (3.0 ±â€Š1.9), social activities and relationships (10.7 ±â€Š5.8) and emotional function (9.2 ±â€Š5.0) classified moderate-to-poor state. In contrast with treatment satisfaction (4.8 ±â€Š1.6), treatment difficulties (9.5 ±â€Š3.4) and mental health (6.2 ±â€Š3.3), those were all in moderate-to-good state. Patients with higher education levels had better quality of life in the area of anxiety (P = 0.034), mental health (P = 0.007), social activities and personal relationships (P = 0.002). In general, poor quality of life of patients was 13.1%, 42.9% weak-to-moderate, 38.1% moderate-to-good and 6% good. It was shown that over 50% of patients had low-to-moderate HRQOL in the domains, such as physical health, daily activities, joint damage, pain, social activities and personal relationships. Efforts are necessary to improve their HRQOL.